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Objective To compare the efficacy and safety of TCZ in monotherapy (TCZMONO) vs. combined with conventional immunosuppressive drugs (TCZCOMBO) in Giant Cell Arteritis (GCA) in a clinical practice scenario. Methods Multicenter study of 134 patients with refractory GCA. Patients on TCZMONO (n = 82) were compared with those on TCZCOMBO (n = 52). Drugs were methotrexate (MTX) (n = 48), azathioprine (n = 3), and leflunomide (n = 1). The main outcomes were: prolonged remission (normalization of clinical and laboratory parameters for at least 6 months) and the number of relapses. Results Patients on TCZCOMBO were younger (68.8 ± 8.0 vs 71.2 ± 9.0 years; p = 0.04), with a trend to a longer GCA duration (median [IQR],18.5 [6.25–34.0] vs. 13.0 [7.75–33.5] months; p = 0.333), higher C-reactive protein (CRP) levels (2.1[1–4.7] vs 1.2 [0.2–2.4] mg/dL; p = 0.003), and more prevalence of extra-cranial large-vessel vasculitis (LVV) (57% vs. 34.1%; p = 0.007). In both groups, rapid and sustained improvement was observed. Despite the longer GCA duration, and the higher CRP levels and prevalence of LVV in the TCZCOMBO, the improvement was similar in both groups at 12 months. Moreover, in the TCZCOMBO group, prolonged remission was significantly higher at 12-month. Relapses and serious adverse events were similar in both groups. Conclusion In clinical practice, TCZ in monotherapy or combined with conventional immunosuppressive agents is effective and safe in patients with GCA. Nevertheless, the addition of immunosuppressive drugs, usually MTX, seems to allow a higher rate of prolonged remission, even in patients with a longer GCA duration, more extra-cranial LVV involvement, and higher acute-phase reactants.

Polymyalgia rheumatica (PMR), a common disease in individuals older than 50 in the western world, is characterized by bilateral inflammatory pain involving the shoulder girdle and less commonly the neck and pelvic girdle. The main goals of the currently available treatment are to induce remission and prevent relapse.This review briefly presents the main epidemiological and clinical features of PMR and discusses in depth both its classical management as well as new therapies used in PMR.In general, patients with isolated PMR experience a rapid response (in less than seven days) to 12.5-25 mg/prednisone/day. Methotrexate is the conventional disease-modifying antirheumatic drug most commonly used for disease management, especially for relapses of the disease. However, this agent often yields a modest effect. Randomized controlled trials do not support the use of antitumor necrosis factor agents in PMR. Several case series and retrospective studies have highlighted the efficacy of the anti-interleukin-6 receptor antibody tocilizumab in PMR. However, controlled trials are needed to fully establish the efficacy of this biologic agent in PMR. The potential beneficial effect of the Janus-kinase inhibitors remains to be determined.

Background:Visual involvement is one of the most feared complication of Giant Cell Arteritis (GCA).Tocilizumab (TCZ) has shown efficacy and safety in large-vessel vasculitis (LVV) including GCA (1-4).Objectives:To assess the efficacy of TCZ to: a) prevent the appearance of new ocular involvement, and b) to improve visual symptoms if present.Methods:Observational, multicenter study of 312 patients with GCA treated with TCZ. Patients were diagnosed with GCA accordingly to a) ACR criteria, and/or b) biopsy of temporal artery, and/or c) presence of LVV by imaging.Patients were divided into two subgroups: a) with, and b) without visual involvement at any time. Visual manifestations were classified as: a) Transient visual loss (TVL) (amaurosis fugax), b) Permanent visual loss (PVL) (longer than 24 hours) (partial or complete; unilateral or bilateral), c) diplopia, and d) blurred vision. Accordingly to visual duration up to TCZ onset, we considered: a) 1-10 days, b) 11-30 days, and c) more than 30 days.Results:We studied 312 (218 women/94 men; mean age73.4±9.6 years). Visual manifestations at any time (before and/or after TCZ) were observed in 78 (25%). In 47 of them visual manifestations were present at TCZ onset, and in the remaining 31 patients had had a complete recovery. Main clinical features of GCA with and without visual involvement are shown in TABLE. Patients with visual involvement were older, with other ischemic complications, and requiring more corticosteroids dose.Table 1.Main features of 312 patients at TCZ onset.OverallN= 312GCA with visual involvement(n= 78)GCA without visual involvement(n=234)pGeneral featuresAge (mean±SD)73.4±9.676.6±8.072.4±9.80.001*Female/Male(% of female), n218/94 (70)47/31 (60)171/63(73)0.046*Time from GCA diagnosis to TCZ onset (months), median [IQR]8 [3-24]5 [1-14]10 [3-24]0.040*Positive TAB, n (%)137/229 (60)33/60 (55)104/169 (61)0.444Ischemic manifestationsVisual involvement, n (%)47 (15)47 (60)00.000*Headache, n (%)166 (53)59 (76)107 (46)0.000*Jaw claudication60 (19)26 (33)34 (14)0.001*Systemic manifestationsFever, n (%)27 (9)8 (10)19 (8)0.743Constitutional syndrome, n (%)115 (37)30 (38)85 (36)0.878PmR, n (%)188 (60)46 (59)142 (61)0.830Acute phase reactantsESR, mm/1st hour, median [IQR]27 [10-50]34.5 [15.2-58]26.0 [10.0-48.0]0.193CRP (mg/dL), median [IQR]1.4 [0.4-3.3]1.5 [0.3-5.5]1.3 [0.4-2.9]0.134Prednisone dose, mg/day, mean±SD22.3±16.627.1±18.620.8±15.60.008*TCZmono/TCZcombo, n (%)211/10157/21154/800.295Follow-up (months), mean±SD28.4±21.825.8±22.429.3±21.60.119After TCZ onset, none patient developed new visual involvement. At TCZ onset 47 patients had the following visual manifestations; PVL (n= 28; unilateral/bilateral; 22/6), TVL (n=15; unilateral/bilateral; 9/6), diplopia (n=2) and blurred vision (n=2).None of the patients with TVL presented new episodes after TCZ onset, while 8 out of 28 patients with PVL experienced partial improvement (FIGURE). The 2 patients with diplopia and 1 of 2 patients with blurred vision improved.Figure 1.Efficacy of TCZ in 47 patients with GCA and visual involvement at TCZ onset.Conclusion:TCZ seems to prevent the appearance of new ocular manifestations. When they are present, TCZ may improve totally TVL and partially PVL.References:[1]Stone JH, et al. N Engl J Med. 2017; 377: 317-28.[2]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019;49:126-35. https://doi.org/10.1016/j.semarthrit.2019.01.003.[3]Prieto Peña D et al. Clin Exp Rheumatol 2020 Nov 27. PMID: 33253103.[4]Loricera J, et al. Clin Exp Rheumatol 2016; 34:S44-53. PMID: 27050507.Disclosure of Interests:Lara Sanchez-Bilbao: None declared, Javier Loricera: None declared, Vicente Aldasoro: None declared, Rafael Melero: None declared, Santos Castañeda: None declared, Olga Maiz: None declared, Clara Moriano: None declared, Ignacio Villa-Blanco: None declared, Eztizen Labrador-Sánchez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eva Galíndez-Agirregoikoa: None declared, Eva Perez-Pampín: None declared, Andrea García-Valle: None declared, Cristina Campos Fernández: None declared, Juan Ramón De Dios: None declared, Carlota Laura Iñíguez: None declared, José Luis Andréu Sánchez: None declared, Julio Sánchez: None declared, Monica Calderón-Goercke: None declared, Miguel A González-Gay Speakers bureau: Abbvie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Consultant of: Abbvie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Grant/research support from: Abbvie, MSD, Jansen and Roche, Ricardo Blanco Speakers bureau: Abbvie, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Consultant of: Abbvie, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Grant/research support from: Abbvie, MSD and Roche

Background:Tocilizumab (TCZ) has shown promising results in the treatment of inflammatory ocular disease, especially in uveitis (1-3). Graves orbitopathy (GO) is the most common complication of Graves’ disease (GD). Conventional immunosuppressive drugs are not always effective or well tolerated. TCZ may be useful in the treatment of GO.Objectives:To assess the efficacy of TCZ in corticoid-resistant GO.Methods:Multicenter open study of corticoid-resistant GO. We measured clinical activity using the clinical activity score (CAS). CAS evaluates 10 different ocular items, ranging from 0 (no symptoms) to 10. We defined remission as the presence of CAS ≤ 3.Results:We studied 48 (95 eyes) patients (TABLE). Besides oral corticosteroids, they had received iv Methylprednisolone (n=43), methimazole (n=20), selenium (n=11) or radioactive iodine (n=5). According to the classification of severity (EUGOGO group), before of TCZ onset our patients had severe (n=19) or moderate (n=29) disease. TCZ was used in monotherapy (n=45) or combined with methotrexate (n=2) or azathioprine (n=1) at a dose of 8 mg/kg/iv/4 weeks (n=43) or 162 mg/sc/week (n=5). TCZ yielded rapid and maintained improvement and most patients achieved remission (FIGURE).TABLE:Number of patients/eyes affected, n/n48/96Age, mean (SD), years50.96 (11.78)Sex, men/women, n/n (%)10/38 (20.8 / 79.2)Regimen of TCZ therapyMonotherapy/combined treatment, n (%)45/3 (93.8/6.2)• AZA1 (2.1)• MTX2 (4.2)TCZ dosage, n (%)• 8 mg/kg/iv/4 weeks43 (89.6)• 162 mg/sc/week5 (10.4)Follow-up on TCZ therapy, mean (SD), months16.05±2.06• Remission, n (%)72 (75.8)• Discontinuation treatment, n (%)29 (60.4) • Remission25 (86.2) • Inefficacy4 (13.8) • Side effects0FIGURE:After a mean follow-up of 16.1±2.1 months, most patients experienced ocular improvement, with TCZ withdrawal in 29 cases due to remission (n=25) or inefficacy (n=4). Only 5 relevant adverse events were observed (neutropenia, external otitis, otitis media, costal osteitis and gingival hyperplasia). None of these events resulted in discontinuation of the treatment.Conclusion:TCZ appears to be an effective and safe treatment in corticoid-resistant GO.References:[1] Santos-Gómez M, et al. Clin Exp Rheumatol 2016; 34 Suppl 102(6):34-40[2] Calvo-Río V, et al. Clin Exp Rheumatol. 2014; 32 (4 Suppl 84): S54-7[3] Vegas-Revenga N et al. Am J Ophthalmol. 2019 Apr; 200:85-94.Disclosure of Interests:Lara Sanchez-Bilbao Grant/research support from: Pfizer, David Martinez-Lopez: None declared, Belén Atienza-Mateo: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Vanesa Calvo-Río Grant/research support from: Abbvie, Lilly, UCB, MSD, Cellgene, Speakers bureau: Abbvie, Lilly, UCB, MSD, Cellgene, Rosalía Demetrio-Pablo: None declared, Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Iñigo González-Mazón: None declared, Elia Valls-Pascual Grant/research support from: Roche, Novartis, and AbbVie, Speakers bureau: AbbVie, Lilly, Pfizer, MSD, Novartis, Janssen, Bristol Myers Squibb, UCB Pharma, Beatriz Valls-Espinosa: None declared, Olga Maiz-Alonso: None declared, Ana Blanco Speakers bureau: Abbvie, Ignacio Torre-Salaberri: None declared, Verónica Rodriguez-Mendez: None declared, Ángel García-Aparicio: None declared, Raúl Veroz González: None declared, Vega Jovani: None declared, Diana Peiteado Grant/research support from: AbbVie, Lilly, MSD, and Roche, Speakers bureau: AbbVie, Roche, and MSD, Santos Castañeda: None declared, Margarita Sánchez-Orgaz: None declared, Eva Tomero Muriel: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Valvanera Pinillos: None declared, Elena Aurrecoechea: None declared, Ángel Mora: None declared, Arantxa Conesa: None declared, Manuel Fernández: None declared, J. Antonio Troyano: None declared, Marcelino Revenga: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Background:Rheumatoid arthritis is an autoimmune disorder in which patients have an increased risk of developement of serious infections. This risk may be augmented due to RA itself and to immunosuppressive drugs, specially biologic therapy. Vaccination programs may change this condition.Objectives:Our aim in this study was to evaluate the incidence of serious infections in a vaccinate RA patients cohort.Methods:Prospective study of 401 patients diagnosed with RA who were invited to participate in the vaccination program of the Preventive Medicine department of our hospital from October 2011 to October 2016. The follow up was made until June 2017 with a minimun follow-up period of 8 months and maximun of 5.5 years. Serious infections were defined as those that required hospitalization or at least one dose of intravenous antibiotic treatement at emergency room. Information was retrieved from dthe hospital records.Only 7 patients refused vaccination (2%). Information was not obtained in 4 of the remaining 394 patients. Therefore, these 4 patients were not incuded in the assessment.Survival análisis was assessed by Kaplan-Meier method.Results:We finally studied 390 patients (307♀/83♂) mean age±SD 61,28 ± 12,9 years that participate in the vaccination program and followed-up. The main features at the time of vaccination were: median disease duration (4years), positive rheumatoid factor (56,7%), subcutaneous nodules (4.9%), erosive arthritis (36.9%), pulmonary fibrosis (3.8%), secondary Sjögren syndrome (5.1%), other extraartocular manifestations (14.6%) and rheumatoid vasculitis (5.6%) Most patients had received imunosuppressive drugs before the vaccination program. The most frequently used were systemic corticosteroids (n=228), methotrexate (n=362) and biologic agents (40.3%).During the follow-up, 42 patients (10.7%) had required hospital admissions due to infections, 17 of them were severe respiratory infections (4.35%). The remaining 25 admissions were in the setting of urinary tract infections (n=12), intraabdominal infections (7), skin and soft tissues (12) and articular (1). Also 12 of these patients had a zoster herpes.Afeter a median follow-up of 1061,89 ± 417 days, the incidence of serious infection, with a CI (95%), was 4.00 (2.95-5.41) for 100 patients yearly. Concerning to admissions due to serious respiratory infections, with a CI (95%), was 1.55 (0.9-2.47) for 100 patients yearly.Images 1 and 2.Image 1.Survival analysis on serious infectionsImage 1.Survival analysis on serious respiratory infectionsConclusion:In this stydy we can concluded that our RA vaccinated patients present a dicrease of the incidence of serious infeccions, similar to other published cohorts. The incidence of serious respiratory infections shows a dicrease even lower to other published cohorts. The vaccination program seems to be effective to prevent hospital admissions due to infections.Disclosure of Interests:Lucia Domínguez: None declared, Paz Rodriguez Cundin: None declared, Vanesa Calvo-Río Grant/research support from: MSD and Roche, Speakers bureau: AbbVie, Lilly, Celgene, Grünenthal, UCB Pharma, Nuria Vegas-Revenga Grant/research support from: AbbVie, Roche, Pfizer, Lilly, Gebro Pharma, MSD, Novartis, Bristol-Myers, Janssen, and Celgene, Virginia Portilla: None declared, Francisco Manuel Antolin-Juarez: None declared, Maria Henar Rebollo Rodriguez: None declared, Alfonso Corrales Speakers bureau: Abbvie, Natalia Palmou-Fontana: None declared, D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their risk of morbidity and mortality. However, little is known about the prevalence of AD in patients diagnosed with an ILD. In this prospective study, we determined the spectrum of ILD associated with AD (AD-ILD) among patients sent for assessment to a single clinic of ILD and lung transplantation from a referral center between May 2016 and December 2019. ILD diagnosis was made by pneumologists based on clinical and radiological findings and pulmonary function test abnormalities. All patients with ILD were also assessed by experienced rheumatologists. During the period of assessment, 338 patients were diagnosed with ILD. Among them, 32.8% fulfilled definitions for an AD. Most cases with AD-ILD had a diagnosis of rheumatoid arthritis (27.0%), systemic sclerosis (26.1%) or anti-synthetase syndrome (17.1%). Interestingly, 18% of the patients with AD-ILD were diagnosed as having an interstitial pneumonia with autoimmune features. Antinuclear antibodies and non-specific interstitial pneumonia were the most frequent positive autoantibodies and radiological pattern found in AD-ILD patients, respectively. In conclusion, our study indicates that a high number of ILD patients have a related AD. Consequently, close collaboration among rheumatologists and pneumologists is needed. This research received no external funding. SR-M is supported by funds of the RETICS Program (RD16/0012/0009) (Instituto de Salud Carlos III, co-funded by the European Regional Development Fund).

Giant cell arteritis (GCA) is the most common vasculitis in individuals older than 50 years in Western countries. In addition to the typical pattern of cranial ischemic manifestations, large vessel vasculitis (LVV) involvement has emerged as a common feature of GCA. Patients with predominant LVV manifestations differ from those with the cranial pattern. They are usually affected at a younger age and often have nonspecific manifestations such as constitutional syndrome, fever of unknown origin, or refractory/atypical polymyalgia rheumatica (PMR). In these patients, cranial manifestations are often absent. Furthermore, patients with isolated PMR should be followed up because of the potential risk of severe vascular complications in the setting of an underlying GCA. Whereas temporal artery biopsy and/or color duplex ultrasound of the temporal arteries is useful for the diagnosis of cranial GCA, Doppler sonography of the subclavian and axillary arteries, fluorine-18-fluorodeoxyglucose-positron emission tomography/computed tomography, magnetic resonance, and computed tomography-angiography are very useful to identify the presence of LVV, and they may play a potential role in the follow-up of these patients.

Background Lupus nephritis (LN) is a severe complication of systemic lupus erythematosus (SLE), affecting up to 30-40% of patients. Unfortunately, approximately 10-20% of LN develop end stage renal disease (ESRD) and need replacement therapy. Renal transplantation may be a good option. However, concerns about LN recurrence after renal transplantation have been reported. Objectives In a series of patients with first renal transplantation due to LN our aim was to assess a) long-term post-transplant survival and, b) comparison of post-transplant survival with a control group due to a non-autoimmune nephropathy, the polycystic kidney disease (PCKD). Methods We study two groups of patients with first renal transplantation: a) LN and b) control group with PCKD. All these patients were transplanted in a single reference University Hospital. The main outcome variables were a) graft and patient survival up to 20 years and b) evolution of renal function (serum creatinine and proteinuria) in the first 5 years of follow-up. Cumulative survival rates after transplantation were estimated by the Kaplan-Meier method and compared between groups using the log-rank test. Mann-Whitney test was used to compare quantitative variables and chi2/Fisher’s exact test for qualitative variables. Results We included a total of 53 patients with renal transplant; a) LN group (n=21), b) PCKD group (n=32). No significant differences at baseline were observed between the 2 groups regarding sex and cardiovascular risk factors. Significant differences were found in terms of age at kidney transplantation, with a mean of 39.80±11.27 years in LN group and 46.59±5.01 years in the PCKD group (p=0.004). Renal biopsy had been performed in 16 patients with LN: type II LN (25%), type III (25%) and type IV (50%), (according to the World Health Organization and International Society of Nephrology/Renal Pathology Society classification). From 48 patients (of 53) in which a renal biopsy was performed during the first-year post-transplant, rejection was found in 21 patients (43.7%) without significant differences between the 2 groups (p=0.444). The evolution of serum creatinine and proteinuria after renal transplantation is shown in TABLE-1. Regarding serum creatinine, significant differences were found in creatinine levels only at the 6th month post-transplant (p=0.032) with no differences in the following measurements. In LN group, 3 patients (14.3%) developed a lupus flare: 2 cases presented as extrarenal disease and only 1 case with histological recurrence in the graft. No significant differences were found in terms of patient or graft survival between the two groups in 20 years of follow-up (Figures 1 and 2). Conclusion Despite concerns about LN recurrence after renal transplantation, data obtained in our sample indicate that this procedure as a safe alternative therapy for ESRD in this population and can provide a long-term survival. *p Disclosure of Interests Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, Marina de Cos-Gomez: None declared, Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Juan Carlos Ruiz-San Millan: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Non ischaemic optic neuritis (NION) is a severe inflammation of the optic nerve that may lead to blindness. It can be primary or associated to immune mediated inflammatory diseases (IMIDs). The treatment of the NION is based on systemic corticosteroids and conventional immunosuppressive drugs. Objectives To assess the efficacy of the biological treatment in refractory NION to conventional treatment. Methods Multicenter study of 12 patients diagnosed with NION refractory to systemic corticosteroids and at least one conventional immunosuppressive drug. The main outcomes were visual acuity (VA) and optical coherence tomography (OCT) of the optic nerve and the ganglionar cells. Comparisons were made between baseline and the 1st week, 1st and 6th month and 1st year. (STATISTICA, StatSoft Inc. Tulsa, Oklahoma, USA). Results We studied 12 patients (19 affected eyes) (5 men/7 women); mean age of 29.8 ±12.9 years. The underlying diseases were systemic lupus erythematosus (n=1), neuromyelitis optica (n=1), neuroretinitis (n=1), relapsing polychondritis (n=1), pars planitis (n=1), Behcet’s disease (n=2) and idiopathic (n=5). Before biological treatment and besides oral corticosteroids, patients had received intravenous (IV) methylprednisolone boluses (n=9), cyclosporine A (n=1), cyclophosphamide (n=2), mycophenolate (n=2), hydroxychloroquine (n=1), methotrexate (n=8) and azathioprine (n=5). Biological treatment was bases on rituximab (n=2) (2 IV, doses of 1 g/every 2 weeks and every 6 moths), adalimumab (n=5) (40 mg/1-2 week), tocilizumab (n=4) (8 mg/kg/2-4 weeks) and infliximab (n=3) (5 mg/kg at 0, 2 and 6 week and then every 8 weeks). The characteristics of the 12 patients are shown in the TABLE. After biological treatment we observed an improvement in the ocular parameters: VA [0.66±0.32 to 0.84±0.29; p= 0.03], OCT of the optic nerve [123.20±58.28 to 190.54±175.38; p= 0.11], and OCT of the ganglionar cells [369.55±137.37 to 270.67±23.21; p= 0.03] at one year. After a mean follow-up of 29.09 ±19.23 months, there were no severe adverse effects. Conclusion Biologic therapy in NION idiopathic or associated to IMIDs, refractory to conventional treatment, seems to be effective. Disclosure of Interests D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Vanesa Calvo-Rio: None declared, Olga Maiz-Alonso Speakers bureau: Pfizer, Ana Blanco: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Esther Vicente: None declared, Susana Romero-Yuste: None declared, Rosalia Demetrio-Pablo: None declared, ANA URRUTICOECHEA-ARANA: None declared, Jose L. Garcia-Serrano: None declared, Jose Luis Callejas-Rubio: None declared, Norberto Ortego: None declared, Julio Sanchez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Fluorine-18-fluorodeoxyglucose (18F-FDG) PET/CT has been proposed as a promising tool for assessing both musculoskeletal and vascular involvement in patients with polymyalgia rheumatica (PMR). Glucocorticoids (GC) may decrease the intensity of 18F-FDG uptake. Therefore, performance of PET/CT before steroid therapy is recommended. However, in many patients with PMR, large vessel vasculitis (LVV) is precisely suspected because of steroid resistance after a long-term treatment with GC 1. Objectives Our aim was to assess the influence of long-term medium-dose treatment on 18F-FDG uptake to discern if 18F FDG PET/CT could be useful to evaluate musculoskeletal and vascular involvement in patients under treatment with GC. Methods Single-center study of 75 patients with PMR diagnosis based on 2012 EULAR/ACR criteria. All patients underwent a PET/CT scan due to LVV suspicion based on the presence of atypical symptoms and/or persistent symptoms despite steroid therapy. We considered two groups: a) Steroid-naive PMR patients. b) Steroid-resistant PMR patients. Both musculoskeletal and vascular 18F-FDG uptake was assessed. The statistical analysis was performed with SPSS. Student’s t test or Mann-Whitney U test was used to compare continuous variables, and Chi-squared test or Fisher’s exact test for categorical variables as appropriate. Results We evaluated 75 patients, 27 men and 48 women (mean age ± SD: 68.2 ± 10.7 years). PET/CT was performed in 14 steroid-naive PMR patients (18.7%) and 61 steroid-resistant PMR patients (81.3%). Patients under steroid treatment had received a median dose of Prednisone of 10.0 [5.0-15.0] mg/day during 9.0 [2.0-22.0] months. Vascular 18F-FDG uptake was more frequently detected in steroid-naive patients. In regard with musculoskeletal 18F-FDG uptake, no statistically significant differences were seen between both groups (TABLE). Conclusion Vascular 18F-FDG uptake detection was higher in PMR patients with LVV suspicion who had never received GC. However, PET/CT was also useful to detect vascular involvement in most of the patients under a long-term medium-dose steroid treatment. Reference [1] D. Prieto-Pena, I. Martinez-Rodriguez, J. Loricera, et al. Predictors of positive (18)F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica. Semin Arthritis Rheum; 2018): Doi: 10.1016/j.semarthrit.2018.05.007. [Epub ahead of print] Disclosure of Interests D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Isabel Martinez-Rodriguez: None declared, Jose Ignacio Banzo: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Tocilizumab (TCZ) has been approved for the treatment of Giant Cell Arteritis (GCA). It showed to be effective to induce remission, prevent relapses and decrease the cumulative prednisone dose. However, the glucocorticoids are the mainstay in the acute treatment of GCA. Objectives Our aim was to compare the efficacy and safety of the initial dose of prednisone at the onset of TCZ treatment. Methods Retrospective, multicenter study on 134 patients with GCA in treatment with TCZ. We compared two subgroups of patients according to the initial dose of prednisone at TCZ onset. Clinical efficacy, analytical improvement and safety was studied. Results We studied 134 patients (101 w/33 m) and made a comparative study between 2 groups: a) TCZ and ≤ 15 mg of prednisone; 68 (50.7%) cases, and b) TCZ and > 15 mg of prednisone, 66 (49,3%) patients. It is summarized in TABLE 1. It was no statistical significance according to age, sex and evolution time of disease. In the group receiving > 15 mg of prednisone, the patients presented more visual involvement (p 15 mg of prednisone (p=0.001 and p=0.006, respectively). TABLE 2 summarizes the infections of our patients. Conclusion According with our results, we can conclude that TCZ is equally effective; in terms of prolonged remission and relapses, with doses ≤ 15 mg of prednisone at treatment onset. Being the most important data, the higher risk to develop adverse effects, as well as infections with higher doses of prednisone. References [1] Proven A. Glucocorticoid therapy in giant cell arteritis: duration and adverse outcomes. Arthritis Rheum. 2003; 49:703-8. [2] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Immunosuppressed patients such as Rheumatoid Arthritis (RA) patients have a greater risk (1.5-2 times) of presenting herpes zoster (HZ). Both, the disease itself and the use of immunosuppressive drugs, are involve in this increased risk. Furthermore, in these patients is more frequent a disseminated presentation of zoster involving several dermatomes. Objectives In a series of RA patients our aim was analyzed HZ infections and to assess a) HZ prevalence and b) HZ general features Methods Prospective Single center study of 390 RA patients included in the vaccination program of the Preventive and Rheumatology department of our hospital between october 2011 and october 2016. The follow-up was made until December 2017. HZ vaccination is not included in our program. RA was diagnosed according to the ACR/EULAR 2010 proposed criteria (Arthritis Rheum 2010; 62: 2569–2581) The diagnose of HZ was made according to the clinical manifestations and was confirmed by a dermatologist. These manifestations were characteristic skin rash and blisters, paresthesia and local pain, in one (localized) or more dermatomes (generalized) Results We studied 390 patients (307♀/83♂), average age 61.28±12.9 years that were included in the vaccination program and followed-up. HZ infection was observed in 12 of 390 (3.07%) in the follow-up (TABLE). The 12 RA patients (11 women/1 man) with a mean±SD age of 67.5±11.67. More than half of patients, 7 (58.33%) were taking corticosteroids. 8 patients (66.66%) were receiving conventional disease modifying drugs (DMARDs) methotrexate (33.33%), leflunomide (16.66%) and hydroxychloroquine (16.66%). Besides corticosteroids and conventional DMARDs, 7 patients (58.33%) were in treatment with biologic drugs, tocilizumab (n=2), etanercept (n=2), adalimumab (n=2), and rituximab (n=1). Conclusion Herpes zoster is a relative frequent viral infection in RA patients non-vaccine for HZ. The female sex, older age, more aggressive RA and treatment with corticosteroids were more frequent. Probably in this group of patients HZ vaccination may be useful. Abbreviatures: MTX: methotrexate, LFN: leflunomide, HCQ: hydroxicloroquine, TCZ: tocilizumbas, ADA: adalimumab, ETN: etanercept Disclosure of Interests: Lucia Dominguez: None declared, Vanesa Calvo-Rio: None declared, Paz Rodriguez-Cundin: None declared, Virginia Portilla: None declared, Nuria Vegas-Revenga: None declared, Francisco Manuel Antolin-Juarez: None declared, Maria Henar Rebollo Rodriguez: None declared, Alfonso Corrales: None declared, D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background In Giant Cell Arteritis (GCA) two dominant cytokine clusters have been linked to disease activity, IL-6 – IL-17 axis (Th17) and IL-12 – IFN γ axis (Th1). The first one related to systemic symptoms and the second route responsible for ischemic symptoms. Tocilizumab (TCZ) performs its effect mainly by inhibiting Th17 axis and terminally Th1 route. Objectives Our aim was to evaluate the effect of TCZ on ischemic and systemic symptoms throughout the follow-up. Methods Retrospective, multicenter study of 134 patients diagnosed of GCA on treatment with TCZ. We evaluate the efficacy of TCZ by improving ischemic (visual involvement, headache, jaw claudication) and systemic symptoms (fever, constitutional syndrome, polymyalgia rheumatica (PMR)). Results We evaluated 134 patients (101 w/33 m) and its main symptoms at TCZ onset, TABLE. 73 (54.5%) patients presented PMR followed by headache in 70 (52.2%) cases, constitutional syndrome in 31 (23.1%) and visual involvement in 28 (20.9%) patients. After one month of treatment there was an important clinical improvement, persisting in 13,6% of patients PMR, 10.6% headache and 10.6% visual involvement. Throughout the follow-up, the improvement of ischemic symptoms was slower. At month 12, in 5.6% (4) of patients persisted with visual impairment, and 2.8% (2) patients presented headache and constitutional syndrome. However, the analytical improvement was statistically significant from the first month and sustained during follow-up. Conclusion According to the results of our study, we can conclude that in clinical practice, ischemic symptoms take longer to improve than systemic symptoms; being visual affectation the most frequent symptom after 12 months of follow-up. References [1] Soriano A, Muratore F, Pipitone N, Boiardi L, Cimino L, Salvarani C. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol. 2017; 13:476-84. [2] Ciccia F, Rizzo A, Ferrante A, Guggino G, Croci S, Cavazza A, et al. New insights into the pathogenesis of giant cell arteritis. Autoimmun Rev. 2017; 16:675-83. [3] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019Jan5. pii: S0049-0172(18)30571-7. Doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Giant cell arteritis (GCA) can be refractory to corticosteroid therapy. Tocilizumab (TCZ) has been approved in the treatment of GCA. There are no studies comparing the efficacy and safety when using TCZ as monotherapy or in combination with conventional immunosuppressive drugs in GCA. Objectives Our aim was to compare efficacy and safety of TCZ combined or in monotherapy in GCA. Methods Multicenter study on 134 patients with refractory GCA who received TCZ therapy as monotherapy or combined with conventional immunosuppressants. Prolonged remission, absence of clinical symptoms and signs and normalization of the acute phase reactants for at least 6 months. Relapse, recurrence of signs or symptoms of GCA and/or ESR >20 mm/h in men or >25 mm/h in women, and/or serum CRP >0.5 mg/dL related to GCA, both before and after starting TCZ therapy. Results We evaluated 134 patients (101 w/33 m) with a mean age of 73.0±8.8 years. TCZ was prescribed as monotherapy in 82 (62.2%) cases and combined with conventional immunosuppressants in 52 (38.8%) patients: MTX (n=48), AZA (n=3), and LFN (n=1). A comparative study between both groups is summarized in TABLE. Patients who received combined TCZ were younger and had a higher C-reactive protein (CRP) and a higher presence of aortitis in imaging techniques. After TCZ was started, prolonged remission was reached with combined therapy (statistical significance at 12 and 24 months). The corticosteroids sparing effect was similar in both groups. And in terms of side effects no significant difference was seen between TCZ as monotherapy or combined with conventional immunosuppressants. Conclusion Patients receiving combined conventional immunosuppressants with TCZ in the clinical practice study showed a higher prolonged remission. The incidence of serious infections and/or relevant adverse events was not affected according to the treatment. As well as the corticoid-sparing effect was achieved in the same way in both groups. References [1] Goercke .Calderon-M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] [2] Loricera J et al. Semin Arthritis Rheum.2015;44:717-723

Background: Giant cell arteritis (GCA) is a systemic vasculitis that affecting large and medium vessels, mainly the temporal artery and other arteries of extracranial localization (1). Objectives: A series of 24 patients with clinical suspicion of GCA proved by a positive temporal artery biopsy (TAB) has been reviewed. Our aim was to asses a) histologic inflammatory patterns in the TAB and b) their possible correlation with analytical and clinical data. Methods: Prospective study of 24 patients with positive TAB between January of 2016 until January of 2018. Clinical data, included: age, sex, symtomatology (craneal, visual or systemic manifestations) and physical examination of the temporal artery. The analytical data registered were: ESR, CRP and haemoglobin. Furthermore, it has been considered if patients were receiving treatment with corticosteroids at the time of the biopsy. Positive biopsies were classified into four categories: a) inflammation limited exclusively to the small vessels of the adventitia (SVV); b) vasa vasorum vasculitis (VVV); c) inflammation limited to the adventitia (ILA); and d) transmural inflammation (TMI). The presence of giant cells, thrombosis and distrophic calcification was also noted. Results: We included a total of 24 patients with positive TAB: a) 19 patients (79.16%) presented TMI and b) 5 patients (20.84%) presented ILA. There were no cases with SVV or VVV. No significant differences at baseline were observed between the 2 groups regarding sex and age, with a mean of 79.6 ± 7.2 years in TMI group and 78.8 ± 7.3 years in ILA group (p = 0.83). There were no significant differences between the correlation of the clinical and analytical data and the 2 patterns of inflammation observed in the biopsy (TABLE-1). Regarding the treatment at the time of performing the BAT, 16 (of 19) patients in the group with TMI (84.2%) and 4 (of 5) patients in the group with ILA (80%) were receiving treatment with oral glucocorticoid (Prednisone). No significant differences between the two groups were found in terms of treatment duration prior to BAT and nor in the dose recived (TABLE-2). Conclusion: In our sample, we didn’t observe differences between the patterns of inflammation and the analytical and clinical data. It is important to recognise these patterns of inflammation in the TAB to avoid false negatives. References 1- MA Gonzalez-Gay, T Pina, D Prieto-Pena, M Calderon-Goercke, R Blanco, S Castaneda (2018) Current and emerging diagnosis tools and therapeutics for giant cell arteritis,Expert Review of Clinical Immunology, 14:7, 593-605, DOI: 10.1080/1744666X.2018.1485491. Disclosure of interests: Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Jose Luis Martin-Varillas: None declared, Belen atienza-Mateo: None declared, C. Gonzalez-Vela: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco Grant/research support from: abbvie, MSD, and Roche, Consultant for: abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Graves’ orbitopathy (GO) is the most common and important extrathyroidal manifestation of Graves’ disease. Corticostereoids and conventional immunosuppressors are not always effective or well tolerated. The IL-6 receptor antibody tocilizumab (TCZ) has demonstrated efficacy in the treatment of this pathology. Objectives To assess the efficacy of TCZ in refractory thyroid associated orbitopathy (TAO) due to Grave’s disease. Methods Multicenter study of 46 patients with TAO refractory to conventional immunosuppressive therapy. Results We studied 46 patients (85 eyes) (37 women/9 men); mean age at diagnosis 49.2±11.8 years. Besides oral corticosteroids, before the onset of TCZ patients had been treated with pulses of iv methylprednisolone (42), radioactive iodine (4), methotrexate (2) and other drugs (selenium in 11 cases, methimazole in 8, leflunomide in 1 and azathioprine in 1). 7 patients underwent ocular urgent decompressive surgery. According to the classification of severity of the EUGOGO group (European Group on Graves’ Orbitopathy) using the clinical activity score (CAS), before TCZ onset patients whose data were available had severe (27 eyes) or moderate (34 eyes) disease. Moreover, patients presented exophthalmos (53 eyes), strabismus (37 eyes), muscle fibrosis (38 eyes) and dysthyroid optic neuropathy (10 eyes). TCZ was used in monotherapy (43) or combined with methotrexate (2) or azathioprine (1) at 8 mg/kg/iv/4 w (41) or 162 mg/sc/w (5). TCZ yielded rapid and maintained improvement in all ocular parameters as shown in Figures. After a mean of 7.42±6.41 months using TCZ and a mean follow-up of 16.47±11.99 months, all patients experienced ocular improvement, with TCZ withdrawal in 28 cases due to complete remission (10), improvement (12) or stability of ocular inflammation (3), inefficacy (2) and total thyroidectomy (1). Only 5 relevant adverse effects were observed (neutropenia, external otitis, otitis media, costal osteitis and gingival hyperplasia, 1 each). Conclusion TCZ appears to be a useful and secure option in GO treatment. References [1] Bartalena L, et al. Consensus statement of the European group on Graves’ orbitopathy (EUGOGO) on management of Graves’ orbitopathy. Thyroid. 2008; 18 (3:333-346). [2] Russell DJ, et al. Tocilizumab as a steroid sparing agent for the treatment of Graves’ orbitopathy. Am J Ophthalmol Case Rep. 2017 Jul 8;7:146-148. Disclosure of Interests Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Vanesa Calvo-Rio: None declared, Rosalia Demetrio-Pablo: None declared, Elia Valls-Pascual: None declared, Beatriz Valls-Espinosa: None declared, Olga Maiz-Alonso Speakers bureau: Pfizer, Ana Blanco: None declared, Ignacio Torre-Salaberri: None declared, Veronica Rodriguez-Mendez: None declared, Angel Garcia-Aparicio: None declared, Raul Veroz Gonzalez: None declared, Vega Jovani: None declared, Diana Peiteado: None declared, Margarita Sanchez Orgaz: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Eva Tomero: None declared, Francisco J. Toyos Saenz de Miera: None declared, Valvanera Pinillos: None declared, Elena Aurrecoechea: None declared, Angel Mora: None declared, Arantxa Conesa: None declared, Manuel Fernandez: None declared, J. Antonio Troyano: None declared, Inigo Gonzalez-Mazon: None declared, Lara Sanchez Bilbao: None declared, D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco: None declared

Background: Sarcoidosis is a multisystemic inflammatory disease characterized by non-caseating epitheliod granulomas that can affect any organ system. The three most frequency affected organs are lung, skin and eyes. Ocular involvement is the presenting symptom in approximately 20-30% and can involve any part of the eye and its anexal tissues. Sarcoidosis may cause uveitis, conjunctivitis, episcleritis/scleritis, optical nerve disease and orbital inflammation. Objectives: To analyze the prevalence of ocular involvement is systemic sarcoidosis, the clinical patterns and their correlation with the International Workshop on Ocular Sarcoidosis (IWOS) criteria. These criteria classify ocular sarcoidosis as definite, presumed, probable and possible, according to some ophthalmological and analytical findings. They are especially useful if a biopsy is not obtained or it is negative. Methods: Retrospective study of patients admitted to a single reference University Hospital between 1999 and 2019 with diagnosis of sarcoidosis. Clinical findings, demographics features, anatomic location and IWOS intraocular signs were recorded. We also collected serum angiotensin converting enzyme (ACE), liver enzyme test, chest radiography, chest computed tomography scan, treatment and biopsy if performed. Results: We selected patients with ocular inflammation from a cohort of 381 patients with sarcoidosis (n=50, 13%). Most of the cases were women (54%) and median age was 45.5±16.7 years. In these 50 cases, the most affected organ was lung (60%), followed by skin (28%). Forty patients had uveitis, 32 of them with ocular symptoms. Thirty-nine out of 50 patients (78%) met one of the 4 IWOS diagnostic categories: 22 with definite (44%), 13 presumed (26%) and 4 with possible (8%) sarcoidosis. Eleven patients did not meet IWOS criteria. The most common ocular signs were bilaterality (44%), snowballs or strings of pearls (38%), mutton-fat KPs (24%), multiple chorioretinal peripheral lesions (14%) and periphlebitis (10%). The median value of ACE was 69 U/l. Forty-four patients (88%) received oral corticosteroids, 21 (42%) received methotrexate, 11 (21%) received another conventional immunosuppressor and 11 (21%) a biological treatment. TABLE shows demographic and clinical features. Conclusion: In our population the IWOS Criteria had a sensitivity of 78%. Even though there is no gold standard for diagnosing ocular sarcoidosis yet, IWOS signs can help clinicians suspect it. References: [1] Herbort CP, Rao NA, Mochizuki M; members of Scientific Committee of First International Workshop on Ocular Sarcoidosis. International criteria for the diagnosis of ocular sarcoidosis: results of the first International Workshop On Ocular Sarcoidosis (IWOS). Ocul Immunol Inflamm. 2009May-Jun;17(3):160-9. Disclosure of Interests: None declared

Background Uveitis is one of the major causes of disability of Behcet’s disease (BD). According to the “Expert panel recommendations”, anti-TNF therapy with infliximab (IFX) or adalimumab (ADA) may be considered as first- or second-line therapy for patients with BD-ophthalmic manifestations. Objectives To compare IFX versus ADA as first biologic drug in refractory uveitis due to BD for 1-year period. Methods Multicenter study of BD-associated uveitis refractory to conventional non-biologic treatment. Dosing schedule: IFX 3-5 mg/kg iv at 0, 2 and 6 weeks and then every 4-8 week, and ADA 40 mg/sc/every other week without loading dose. Main comparative outcome measures: safety and efficacy, assessing the intraocular inflammation, macular thickness, visual acuity, degree of immunosuppression load, drug retention, and glucocorticoid-sparing effect. Results 177 patients (316 affected eyes) were included. IFX was used in 103 and ADA in 74. No significant differences at baseline were observed regarding main demographic features, previous therapy and ocular severity. After 1 year of therapy, we observed an improvement in all ocular parameters in IFX vs ADA groups: AC inflammation (78.18% vs 92.31%), vitritis (78.95% vs 93.33%), retinal vasculitis (97% vs 95%), macular thickness (264.89±59.74 vs 250.62±36.85), and BCVA (0.67±0.34 vs 0.81±0.26). Drug withdrawal was observed in 57 (55.33%) of IFX group and in 21 (28.37%) of ADA group. Conclusion After 1 year of therapy in refractory BD-associated uveitis, ADA showed a statistically better outcome than IFX in improvement of BCVA, vitritis and drug retention. References [1] Levy-Clarke G, et al. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014; 121:785-96. [2] Santos-Gomez M, et al. The effect of biologic therapy different from infliximab or adalimumab in patients with refractory uveitis due to Behcet’s disease: results of a multicentre open-label study. Clin Exp Rheumatol 2016; 34 (6 Suppl 102): S34-40 [3] Atienza-Mateo B, et al. Anti-IL6-Receptor Tocilizumab in Refractory Uveitis Associated With Behcet’s Disease. Multicenter Retrospective Study. Rheumatology (Oxford). 2018 May 1;57(5): 856-864. [4] Martin-Varillas JL, et al. Successful Optimization of Adalimumab Therapy in Refractory Uveitis Due to Behcet’s Disease. Ophthalmology. 2018 Mar 27. Disclosure of Interests Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Vanesa Calvo-Rio: None declared, Rosalia Demetrio-Pablo: None declared, Emma Beltran: None declared, Juan Sanchez-Burson: None declared, Marina Mesquida: None declared, Alfredo Adan : None declared, Victoria Hernandez: None declared, Marisa Hernandez-Garfella: None declared, Elia Valls-Pascual: None declared, Lucia Martinez-Costa: None declared, Agusti Sellas-Fernandez: None declared, Miguel Cordero-Coma: None declared, Manuel Diaz-Llopis: None declared, Roberto Gallego: None declared, Jose L. Garcia-Serrano: None declared, Norberto Ortego: None declared, Jose M. Herreras: None declared, Alejandro Fonollosa: None declared, Angel Garcia-Aparicio: None declared, Olga Maiz-Alonso Speakers bureau: Pfizer, Ana Blanco: None declared, Ignacio Torre-Salaberri: None declared, Cruz Fernandez-Espartero: None declared, Vega Jovani: None declared, Diana Peiteado: None declared, Esperanza Pato: None declared, Juan Cruz: None declared, Carlos Fernandez-Cid: None declared, Elena Aurrecoechea: None declared, Miriam Garcia-Arias: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Miguel A. Caracuel-Ruiz: None declared, Carlos A. Montilla-Morales: None declared, Antonio Atanes-Sandoval: None declared, Felix Francisco: None declared, Santos Insua: None declared, Senen Gonzalez-Suarez: None declared, Amalia Sanchez-Andrade: None declared, Fernando Gamero: None declared, Luis F. Linares Ferrando: None declared, Fredeswinda Romero: None declared, A. Javier Garcia-Gonzalez: None declared, RAQUEL ALMODOVAR: None declared, Enrique Minguez: None declared, Carmen Carrasco-Cubero: None declared, Alejandro Olive: None declared, Julio Vazquez: None declared, Oscar Ruiz-Moreno: None declared, Fernando Jimenez-Zorzo: None declared, Javier Manero: None declared, Santiago Munoz Fernandez: None declared, Cristina Fernandez-Carballido: None declared, Esteban Rubio-Romero: None declared, Fred Anton Pages: None declared, Francisco J. Toyos Saenz de Miera: None declared, Myriam Gandia Martinez: None declared, David Diaz Valle: None declared, Francisco J Lopez-Longo: None declared, Joan Miquel Nolla: None declared, Enrique Raya: None declared, Marcelino Revenga: None declared, J. Luis Hernandez: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Inigo Gonzalez-Mazon: None declared, Lara Sanchez Bilbao: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco: None declared

Background Polymyalgia rheumatica (PMR) diagnosis is based on the combination of shoulder and pelvic girdle pain associated with elevated acute phase reactants. Traditionally, Bird et al. 1 and Chuang et al.2 criteria have been used for establishing PMR diagnosis. In 2012 an international working group developed new European League Against Rheumatism (EULAR)/American Collegue of Rheumatology (ACR) classification criteria for PMR.3 Objectives: a) To compare the performance of Bird et al., Chuang et al. and 2012 EULAR/ACR classification criteria for PMR in a single-center study. b) To describe the characteristics of the patients excluded by these criteria. Methods We included 100 patients with new-onset PMR who were consecutively diagnosed over a 7-year period by experienced rheumatologists from a single center. PMR diagnosis was confirmed during a prospective 24-months follow-up after excluding other mimicking conditions. Rheumatoid factor (RF) and anticitrullinated protein antibodies (ACPA) were determined in all patients. Subjects were classified by each of the three different criteria. Results We studied 100 patients (61 women/39 men); mean age 67.1 ± 10.2 years. 96% of the patients fulfilled Bird et. al criteria, 94% fulfilled Chuang et al. criteria and 84% of the patients fulfilled 2012 EULAR/ACR criteria (TABLE 1, 2 and 3). We assessed the characteristics of the 16 patients who did not fulfilled 2012 EULAR/ACR criteria: 6 of them were under 50 years of age (range age 43-48 years). Another 10 patients did not complain of pain/stiffness in the shoulder girdle at any time. However, they had typical inflammatory pain involving the pelvic girdle and elevated acute phase reactants. Conclusion In our single-center experience we found that 2012 EULAR/ACR criteria classified most of our patients. However, patients who presented predominant pelvic girdle affection and those who were under 50 years old despite showing all the rest of clinical and analytical required criteria were excluded. Some of these patients did fulfill Bird and/or Chuang criteria. References [1] Bird HA, et al. Ann Rheum Dis.1979;38:434-9 [2] Chuang T, et al. Ann Intern Med. 1982; 97:672-80 [3] Dasgupta B, et al. Ann Rheum Dis. 2012; 71:484-92 Disclosure of Interests D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Rapidly Progressive Glomerulonephritis (RPGN) is characterized histology by the presence of crescents and clinically by a rapid and severe decline in kidney function. Thus, this entity may lead to an end stage renal disease, with kidney transplantation. RPGN can be primary, without extra-renal involvement (RPGN-renal-limited), or secondary to systemic autoimmune disorders (RPGN-SAD), infectious diseases or drugs. Kidney transplantation in RPGN-SAD may be associated to a worse outcome. Objectives In a series of patients with first transplantation due to RPGN our aim was to assess a) long-term post-transplant survival in RPGN-SAD, b) comparison of post-transplant survival between RPGN-SAD and RPGN-renal-limited and c) comparison of both RPGN (SAD and renal-limited) with a control group of non-immunological disorder, the polycystic kidney disease (PCKD). Methods We study three groups of patients: a) RPGN-SAD due to granulomatosis with polyangiitis (n=3), microscopic polyangiitis (n=6) or Goodpasture syndrome (n=2), b) RPGN-renal-limited and c) control group of patients with PCKD. All these patients were transplanted in a single reference University Hospital. The main outcome variables were a) graft and patient survival up to 20 years and b) evolution of renal function (serum creatinine and proteinuria) in the first 5 years of follow-up. Cumulative survival rates after transplantation were estimated by the Kaplan-Meier method and compared between groups using the log-rank test. Kruskal-Wallis test was used to compare quantitative variables and chi2/Fisher’s exact test for qualitative variables. Results We included a total of 100 patients with renal transplant; a) RPGN-SAD group (n=11), b) RPGN-renal-limited group (n=32), and c) PCKD group (n=57). No significant differences at baseline were observed between the two RPGN groups regarding sex, age and cardiovascular risk factors. Compared to the PCKD group, patients with RPGN presented higher cholesterol levels at the time of the transplant (p=0.041) with no other significant differences. Renal biopsy had been performed in the 43 patients with RPGN: type I (27.9%), type II (4.7%), type III RPGN (41.9%) and 25.6% of patients had not classified RPGN (no immunofluorescence was performed at the time of the biopsy). From 89 patients (of 100) in which a renal biopsy was performed during the first-year post-transplant, rejection was found in 33 patients (37.1%) without significant differences between the 3 groups (5 cases in the RPGN-SAD group, 11 cases in the RPGN-renal-limited group and 17 cases in the PCKD group; p=0.592). The evolution of serum creatinine and the proteinuria after the transplant is shown in TABLES 1-2. There were no significant differences between the three groups in the serum creatinine values during at 1st, 6th, 12th, 36th and 60th months post-transplant. Neither differences were found in terms of graft and patient survival between the 3 groups in 20 years of follow-up (Figures 1 and 2). Conclusion Our study shows similar graft and patient survival as well as renal outcome in renal transplant due to RPGN-SAD and RPGN-renal-limited. These outcomes were also similar in non-immune renal disease. Therefore, renal transplantation could be the best option for patients with end stage renal disease due to RPGN regardless of systemic manifestations. Disclosure of Interests Lara Sanchez Bilbao: None declared, Marina de Cos-Gomez: None declared, Inigo Gonzalez-Mazon: None declared, Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Juan Carlos Ruiz-San Millan: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Recently, based on the GiACTA trial results, weekly subcutaneous Tocilizumab (TCZ) has been approved for the treatment of Giant Cell Arteritis (GCA). It has showed to be effective and safety. Objectives Our aim was to compare the efficacy of TCZ according the route of administration. Methods Multicenter study of 134 GCA patients in treatment with TCZ. It was performed a comparative study between 2 groups according the route of administration of TCZ, intravenous (IV) or subcutaneous (SC). Results We study 134 patients divided in 2 groups: a) IV TCZ, 104 cases and, b) SC TCZ, 30 cases, with a mean age 73.4±8.2 years vs 71.9±10.6 years, respectively (p=0.501). Disease duration, clinical manifestations and acute phase reactants at TCZ onset were similar in both groups with non-statistical difference. 91.7% patients who received SC TCZ achieved prolonged remission after 12 months of treatment (p=0.043). And the glucocorticoid sparing effect of TCZ was greater in the same group, reaching a statistical difference at 3 and 24 months (p=0.017 and p=0.021). Patients under IV TCZ treatment suffered more adverse event during follow up (p=0.043). TABLE 1 and 2 summarizes the comparative study. Conclusion Patients in treatment with SC TCZ, reached prolonged remission after 12 months of treatment and were able to discontinue prednisone dose after 24 months of follow up. The incidence of adverse events was more frequent in the IV TCZ group, without difference in relation to infections. References [1] Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med. 2017; 377:317-28. [2] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Sarcoidosis is a systemic granulomatous disease characterized by the presence of non-necrotizing granulomas in different parenchyma. Objectives To describe demographic, clinical and analytical features in a cohort of patients with Sarcoidosis diagnosis from northern Spain during the last twenty years. Methods Descriptive study of 381 patients diagnosed with sarcoidosis during the period 01/01/1999 to 01/01/2019. Biopsy and/or clinical and compatible imaging tests were required for the diagnosis of sarcoidosis. Demographic parameters, clinical manifestations, complementary tests and treatment were registered. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate. Results 381 patients (192 female/191 male, ratio 1:1), with a mean age 45.5±15.4 years at disease onset, and with 94.8% of Spanish nationality. 33% were smokers at diagnosis and 6.3% had tuberculosis (Table 1). The sarcoidosis incidence rate in our population was 3.3 cases/100000 p/year, similar to other national series1. Most frequent clinical manifestations were as follow: pulmonary symptoms (72.3%), general symptoms (37.3%), skin involvement (31%), joint manifestations (27.9%), ophthalmological manifestations (13.0%), digestive disorders (9.3%), neurological symptoms (6.5%), nephrological (4.7%) and cardiological involvement (1.6%) (Table 1). In addition, 11.5% of the patients had a Lofgren syndrome, 0.5% a Heerdfort syndrome and up to 11.5% had mediastinal adenopathy as an incidental finding in simple chest radiography. A simple chest radiograph was performed in all patients. 83.7% presented abnormal patterns: stage I (41.4%), stage II (32%), stage III (7.6%) and stage IV (3.8%). In addition, 29.9% and 10.5% of patients presented pathological scintigraphy and PET respectively. Biopsy was performed in 81.9%, with a mediastinal approach in 43.3%. (Table 2). After a median follow-up of 11.0 [6.0-17.0] years, we observed that up to 32% of patients never received treatment. In the remaining 68%, the main treatment was oral glucocorticoids with a mean dose of 43.4±19.1 mg/day. Other treatments used were conventional immunosuppressants and biological agents (table 2). Conclusion Results obtained were similar to other national and international series, with the exception of non-predominance of female sex and the highest percentage of ocular involvement in our study. Currently, biological treatment (especially anti-TNF-alpha) is used more frequently. References [1] Manaet al. Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: Cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona, Spain. Medicine (Baltimore). 2017Jul;96(29):e7595. Disclosure of Interests Jose Luis Martin-Varillas: None declared, Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, Raul Fernandez Ramon: None declared, D. Prieto-Pena: None declared, David Martinez-Lopez: None declared, Eva Pena Sainz-Pardo: None declared, Belen Atienza-Mateo: None declared, Monica Calderon-Goercke: None declared, Rosalia Demetrio-Pablo: None declared, Vanesa Calvo-Rio: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Aortitis can be idiopathic or associated with other conditions. It is frequently refractory to conventional immunosuppressive therapy. Tocilizumab (TCZ), an anti-IL-6 receptor antibody seems to be effective and safe. Objectives Our aim was to assess the efficacy and safety of TCZ at short and long follow-up in a series of patients with Aortitis. Methods Retrospective, multicenter study of 79 patients diagnosed of inflammatory aortitis based on imaging techniques (PET/CT, CT angiography and/or MR angiography). Results We study 79 patients (61 w/ 18 m). 59 (74.7%) cases were Aortitis secondary to Giant Cell Arteritis (GCA), while 20 (25.3%) were idiopathic. The mean age was 71±8.5 years vs 64.2±7.1 years, respectively (p=0.001). At time of disease diagnosis more than a half of patients (59.5%) presented as main symptom polymyalgia rheumatica (PMR). Aortitis was diagnosed with PET/CT (71 patients), angioRMN (12 patients) and angioCT (8 patients). Prior to TCZ treatment, 61 (77.2%) patients had received conventional immunosuppressive drugs, 59 (74.7%) of them received MTX. After 24 months of treatment with TCZ, more than 75% of patients reached a prolonged remission in both groups (p=0.527), with only 4% of relapses after the same follow-up period (p=1.000). 40 (50.6%) patients had a control image technique (PET/CT) throughout follow up. 4 (3 secondary to GCA and 1 idiopathic) patients reached a complete improvement in uptake after one year of treatment. Conclusion Our results show that idiopathic aortitis occurs in younger patients compared with aortitis secondary to GCA. TCZ proved to be effective in both pathologies, allowing clinical and analytical improvement, as well as a reduction of corticoid dose, without increasing the risk of relapse. However, the improvement in imaging techniques seems to be slower. Reference [1] Loricera J, Blanco R, Castaneda S, Humbria A, Ortego-Centeno N, Narvaez J, et al. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol. 2014; 32:79-89. Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Francisca Sivera: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Carlos Vazquez: None declared, Pau Lluch: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Jose Luis Martin-Varillas: None declared, Sabela Fernandez: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background: Oral and/or genital aphthous ulcers are the most common symptoms of Behcet’s disease (BD), and are often refractory to conventional treatment. The inhibitor of phosphodiesterase-4 Apremilast (APR) has demonstrated efficacy in the treatment of this manifestations. Objectives: To assess the efficacy and safety of APR in BD patients with oral and/or genital ulcers refractory to conventional treatment. Methods: National multicenter open-label study on 49 BD patients treated with APR at maintained standard dose of 30 mg twice daily, with the initial 5-day titration schedule in 38 cases. The main outcome was achievement of oral and/or genital ulcers remission. Results: We included 49 patients (35 women/14 men), mean age of 44.5±13.4 years. Before APR, all patients had received several systemic conventional and/or biological drugs: oral corticosteroids (n=45), colchicine (n=48), NSAIDs (n=21), methotrexate (n=27), azathioprine (n=23), cyclosporine (n=9), dapsone (n=6), adalimumab (n=12), infliximab (n=8), tocilizumab (n=3), etanercept (n=3), sulfasalazine (n=2), cyclophosphamide (n=2) and/or others (pentoxifylline, thalidomide, mycophenolate mofetil, hydroxychloroquine, golimumab, 1 each). The main clinical symptoms for starting APR were oral (n=18) and genital (n=2) aphthous ulcers or both (n=29). After a mean follow-up of 8.3±6.8 months, most of the patients experienced main clinical improvement and prednisone dose was reduced or discontinued (TABLE). In this period of time, 31 patients developed any side-effect, most of them transitory: nausea (n=12), diarrhea (n=11), dyspepsia (n=9), abdominal pain (n=7), headache (n=5), loss of appetite (n=4), weight loss (n=3), halitosis (n=1), dry mouth (n=1), palpitations (n=1) and/or depression (n=1). Six patients had to reduce the dose to 30 mg/day. APR was discontinued in 11 patients due to: not obtaining the expected improvement (n=5), intense gastrointestinal adverse effects (n=3), desire of pregnancy (n=1), persistent erythema nodosum (n=1) and development of neurological involvement (n=1). Conclusion: Our data show a rapid and maintained improvement with APR in patients with mucocutaneous ulcers of BD refractory to several systemic drugs, including biologic therapy. References: [1] Davatchi F, et al. The International Criteria for Behcet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatology Venereol. 2014;28(3):338–47. [2] Gulen Hatemi, et al. Apremilast for Behcet’s Syndrome — A Phase 2, Placebo-Controlled Study. N Engl J Med 2015; 372:1510-1518. Disclosure of Interests: Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, J. Loricera: None declared, Vanesa Calvo-Rio: None declared, Jenaro Grana: None declared, Gerard Espinosa: None declared, Clara Moriano: None declared, Trinidad Perez-Sandoval: None declared, Manuel Martin-Martinez: None declared, Elvira Diez Alvarez: None declared, Maria Dolores Garcia-Armario: None declared, Esperanza Martinez: None declared, Ivan Castellvi Consultant for: I received fees less than 5000USD as a consultant for Kern and Actelion, Paid instructor for: I received fees less than 2000USD as a instructor for Boehringer -Ingelheim, Novartis and Gebro, Speakers bureau: ND, Patricia Moya: None declared, Francisca Sivera: None declared, Jaime Calvo Consultant for: Bristol-Myers Squibb, Janssen, Celgene, Sanofi Genzyme, Speakers bureau: Bristol-Myers Squibb, Isabel de la Morena Speakers bureau: Abbvie, Celgene, Pfzier, UCB, Ghebro, Roche, Sanofi, Janssen., Francisco Ortiz-Sanjuan: None declared, Jose Andres Roman-Ivorra: None declared, Ana Perez Gomez: None declared, Sergi Heredia: None declared, Alejandro Olive: None declared, Agueda Prior-Espanol: None declared, Carolina Diez: None declared, Juanjo J Alegre-Sancho: None declared, D Ybanez-Garcia: None declared, Angels Martinez-Ferrer: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Ignasi Figueras: None declared, Ana Isabel Turrion : None declared, Susana Romero-Yuste: None declared, Pilar Trenor: None declared, Soledad Ojeda Grant/research support from: AMGEN, Speakers bureau: AMGEN, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Inigo Gonzalez-Mazon: None declared, Lara Sanchez Bilbao: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco: None declared

Background Immune checkpoint blockade therapy (ICBT) currently is one of the most used therapies against cancer. The activation of the immune system can lead to different immune-related adverse events (irAEs), being the rheumatological side effects among the most common. Objectives Our aim was to assess the rheumatological irAEs in patients who received immunotherapy. Methods We set up an observational study of patients treated with Nivolumab and Pembrolizumab (anti-PD1), Atezolizumab (anti-PD-L1) and Ipilimumab (antiCTLA-4) for solid organ tumors. All these patients were followed in a single reference University Hospital from March-2015 up to December-2018. The main outcome was to determinate the incidence of rheumatological irAEs. Results We studied 102 patients (63♂/39♀) with a mean age of 60.6±9.7 with different solid organ tumors. Only 7 patients (6.8%) had a previous diagnosis of an immune-mediated disease: psoriasis (n=2), psoriatic arthritis (1), systemic lupus erythematosus (1), spondyloarthitis (1), rheumatoid arthritis (1) and skin lupus (1). Rheumatological side effects were observed in 15 patients (14.7%): inflammatory arthralgia (8), arthritis (6), myositis (2) and aortitis (1). The time of appearance of the rheumatological irAEs was of 6.36 months (±5.81). From the 7 patients with previous diagnosis of an immune-mediated disease, only 1 patient with psoriasis suffered a worsening of skin symptoms and another one with psoriatic arthritis had a monoarthritis episode. Among the 14 patients who suffered from arthralgia/arthritis, the most frequent pattern was oligoarticular (40%), followed by poly (30%) and monoarticular (30%). The most affected joint was the knee (n=4), followed by wrist (3), hands (3), ankle (2), shoulder (2) and foot (1). Apart from the articular disease, 2 patients suffered from myositis in lower limbs, with weakness of legs and arthralgia in their knees. A patient was diagnosed of aortitis through a PET, although the patient did not have any symptom. Immunology tests with RF, ACCP and ANA were done in 8 out of the 15 patients with musculoskeletal irAEs, being negative in all cases. Interviewing 40 of the patients who received ICBT, 7 of them (17.5%) referred xerophthalmia. 6 of them (15%) had a positive Schrimer test, and 3 (7.5%) had a reduced tear break-up time. None patient met Sjogren criteria. In 6 out of the 15 patients with rheumatological irAEs (all of them with arthralgia/arthritis) the ICBT was removed, temporary in all cases, and could be reintroduced after a mean of 33 ± 18.9 days. 6 patients were treated with NSAIDs and 5 with oral prednisone (2 of them also required intra-articular corticoids). Conclusion Rheumatological irAEs are common in clinical practice. Arthralgia-Arthritis and dry-syndrome are the most frequent adverse events. Disclosure of Interests Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, Rosalia Demetrio-Pablo: None declared, Jose Luis Martin-Varillas: None declared, Marina Delgado Ruiz: None declared, Isabel Bernat Pina: None declared, Belen Atienza-Mateo: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Almudena Garcia Castano: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen DOI: 10.1136/annrheumdis-2019-eular.7386

Background Ocular involvement in Behcet’s disease (BD) is a potential severe and disabling complication. Anti-TNF-α agents have shown an improvement of visual outcome in BD-related uveitis refractory to conventional immunosuppressive (IS) drugs. However, these drugs do not achieve control of intraocular inflammation in all patients or are not well tolerated. Tocilizumab (TCZ) has shown efficacy in different refractory ocular inflammatory diseases. Objectives To assess the efficacy of long-term therapy with TCZ in refractory uveitis associated to extraocular manifestations (EOM) due to BD. Methods Multicenter study of patients with BD refractory to standard systemic treatment. Results We followed up 14 patients (9 men/5 women) (26 affected eyes); mean age 40.8±19.5 years. Pattern of ocular involvement: panuveitis (10; 4 with retinal vasculitis), anterior (3) and posterior (1) uveitis; 8 recurrent and 6 chronic; 9 with cystoid macular edema. At TCZ onset the following EOM were present: oral and/or genital ulcers (10), arthritis (6), folliculitis/pseudofolliculitis (6), erythema nodosum (3), livedo reticularis (1), intestinal affection (1) and neurological involvement (3). Before TCZ, they had received corticosteroids (13 intraocular, 12 oral and 12 iv), conventional IS drugs and biologic agents: methotrexate (11), cyclosporine (8), azathioprine (10), colchicine (1), cyclophosphamide (2), mycophenolate mofetil (1), adalimumab (10), infliximab (6), golimumab (3), canakinumab (1), or etanercept (1). TCZ was used in monotherapy (7) or combined with conventional IS drugs (7) at 8 mg/kg/iv/4 w (11) or 162 mg/sc/w (3). After a mean follow-up of 21.7±14.5 months using TCZ, all patients experienced ocular improvement, with complete remission in 10. However, TCZ was only effective in 5 of the patients with EOM. TABLE shows the evolution of ocular parameters. TCZ had to be withdrawn temporally in 1 case, due to an episode of cellulitis with sepsis, and definitely in 4 cases, due to a severe infusion reaction, arthritis impairment, persistence of oral ulcers or a new episode of uveitis (1 each). Prednisone dose was significantly reduced until suspension in all patients. Conclusion TCZ seams a useful and secure therapy in highly refractory BD-related uveitis. References [1] Atienza-Mateo B, Calvo-Rio V, Beltran E, et al. Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behcet’s disease: multicentre retrospective study. Rheumatology (Oxford). 2018 May 1;57(5):856-864. [2] Calvo-Rio V, de la Hera D, Beltran-Catalan E, et al. Tocilizumab in uveitis refractory to other biologic drugs: a study of 3 cases and a literature review. Clin Exp Rheumatol.2014; 32(4 Suppl 84): S54-7. Disclosure of Interests Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Vanesa Calvo-Rio: None declared, Rosalia Demetrio-Pablo: None declared, Natalia Palmou-Fontana: None declared, J. Loricera: None declared, Emma Beltran: None declared, Marisa Hernandez-Garfella: None declared, Lucia Martinez-Costa: None declared, Elia Valls-Pascual: None declared, Antonio Atanes-Sandoval: None declared, Miguel Cordero-Coma: None declared, Joan Miquel Nolla: None declared, Carmen Carrasco-Cubero: None declared, Julio Sanchez: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco: None declared

Background: Behcet’s disease (BD) is a variable vessel vasculitis and typically presents with mucocutaneous involvement. However, any organ can be affected, being the neurological affectation (neurobehcet, NB) one of the most serious manifestations. Objectives: Our aim was to assess the efficacy and safety of biological therapy as treatment of NB. Methods: We set up a multicenter observational study of 29 patients with NB on treatment with biological therapy (BT). NB diagnosis was made by neuroimaging, CSF analysis and/or suggestive clinical signs of central and/or peripheral nervous system involvement, excluding infectious causes or more prevalent pathology. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate. Results: 29 patients (15♂/14♀) with an average age of 39.6 ± 10.5 years. HLA-B51 was positive in 48.3% of the patients. Table shows the non-neurological manifestations. Regarding the neurological manifestations, 23 patients (79.3%) had parenchymal involvement (hemiparesis (n=6), brainstem involvement (n=1), encephalopathy (n = 4), optic neuropathy (n=3), dysphasia (n=1), polyneuropathy (n=6), cognitive impairment (n=4), and non-steroidal psychosis (n=1), while the remaining 6 patients (20.7%) presented aseptic meningitis as a non-parenchymal affectation (Table). Prior to BT, patients had received the following treatment: oral prednisone (n=27), methylprednisolone bolus (n=9), CsA (n=8), AZA (n=16), MTX (n=14) and mycophenolate (n=2). After a median of 31 [10-60] months since the beginning of the neurological symptoms, the following BT was initiated: infliximab (IFX)(n=17), adalimumab (ADA)(n=7), tocilizumab (TCZ) (n=2), golimumab (GOL) (n=2) and Etanercept (ETN) (n=1). A first switch to ADA was necessary in 8 patients with IFX due to primary failure. In addition, 2 of them needed a second switch to TCZ, getting a partial response. The BT was discontinued in 5 patients, 2 of them for obtaining clinical remission and the remaining 3 for inefficacy. After a median follow-up of 5.4±4.6 years, complete response was obtained in 15 patients, partial response in 11 and no response in the remaining 3. We observed an anaphylactic reaction and psoriasis induced by IFX, without other serious adverse events (Table). Conclusion: BT, especially anti-TNF, seems effective and safe for treatment in NB. Disclosure of Interests: Jose Luis Martin-Varillas: None declared, Inigo Gonzalez-Mazon: None declared, Belen Atienza-Mateo: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Lara Sanchez Bilbao: None declared, Vanesa Calvo-Rio: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Esther Vicente: None declared, Olga Maiz: None declared, Clara Moriano: None declared, Elvira Diez Alvarez: None declared, Jose Luis Andreu Sanchez: None declared, Concepcion Delgado Beltran: None declared, Marta Loredo Martinez: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Angel Ramos Calvo: None declared, Francisca Sivera: None declared, Enrique Raya: None declared, Norberto Ortego: None declared, Jose Luis Callejas-Rubio: None declared, Anahy Brandy-Garcia: None declared, Alejandro Olive: None declared, Sabela Fernandez: None declared, Ricardo Gomez de la Torre: None declared, Ignacio Torre-Salaberri: None declared, Julio Sanchez: None declared, ANA URRUTICOECHEA-ARANA: None declared, Eva Salgado-Perez: None declared, Rafael Melero: None declared, Olga Martinez Gonzalez: None declared, Susana Romero-Yuste: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Giant cell arteritis (GCA) is a large vessel vasculitis with a predisposition for the cranial branches of the external carotid artery. However, aorta and/or its main branches may also be involved. Objectives In a series of patients with GCA who presented extracranial vessel involvement, our aim was to assess a) the vascular territories most frequently affected and b) correlation of a major extension of extracranial vascular involvement with a more severe clinical and analytical features. Methods Multicenter study of 68 patients with GCA who presented a compromise of extracranial vessels confirmed by PET/CT. Visual analysis of vascular uptake was performed on supra-aortic trunks (SAT), aortic arch (AA), thoracic aorta (TA), abdominal aorta (AbA), iliac arteries (IA), lower limb arteries (LLA), and upper limb arteries (ULA). Results We evaluated 68 patients with GCA (51w/17m) with a mean age of 68.0±8.3 years. The vascular territories affected were: TA (n=58, 85.29%), SAT (n=38, 55.88%), AbA (n=28, 41.18%), AA (n=18, 26.47%), LLA (n=17, 25%), IA (n=13, 19.12%) and ULA (n=6, 8.82%). We considered 3 groups according to the number of vascular territories affected: a) 1 or 2 territories, b) 3 or 4 territories and c) 5 or more territories and made a comparative study between this groups. In patients with ≥5 vascular territories affected, we observed a higher baseline ESR, and the most frequent systemic manifestations were polymyalgia rheumatica and constitutional symptoms with statistical significance (TABLE). Distribution of categorical variables was compared by the Pearson Chi-squared test. Quantitative variables were analyzed using the ANOVA test. Conclusion In patients with GCA the involvement of TA is very frequent, followed by the SAT and the AbA. Regarding the laboratory findings, patients with higher levels of ESR presented a major extension of extracranial vascular involvement, as well as presenting PMR and/or constitutional symptoms was also related to more affection of extracranial territories. References [1] Loricera J, Blanco R, Hernaandez JL, et al. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol. 2015; 34: 372-377. [2] Loricera J, Blanco R, Hernaandez JL, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015; 33: S19-31. Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Elena Aurrecoechea: None declared, Ignacio Villa-Blanco: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Vicente Aldasoro: None declared, Noelia Alvarez-Rivas: None declared, Nagore Fernandez-Llanio: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Francisca Sivera: None declared, Eva Galindez: None declared, Roser Solans-Laque: None declared, Susana Romero-Yuste: None declared, Norberto Ortego: None declared, Marcelino Revenga: None declared, Rafael Melero: None declared, Eva Salgado-Perez: None declared, Sabela Fernandez: None declared, Vanesa Calvo-Rio: None declared, Natalia Palmou-Fontana: None declared, Jose Ignacio Banzo: None declared, Isabel Martinez-Rodriguez: None declared, Carmen Gonzalez-Vela: None declared, Raquel Dos-Santos: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Immune checkpoint blockade therapy (ICBT) has shown remarkable benefit in different cancer types. Blockade of intrinsic down-regulators of immunity increases the activity of the immune system, which can lead to different immune-related adverse events (irAEs). Our aim was to assess the immune-related adverse events in patients who received anti-cytotoxic T-lymphocyte antigen 4 (CTLA-4), anti-programmed cell death 1 (PD-1) or anti-programmed cell death ligand 1 (PD-L1). Objectives Our aim was to assess the incidence, treatment and evolution of irAEs due to ICBT. Methods We set up an observational study of patients treated with Nivolumab and Pembrolizumab (anti-PD1), Atezolizumab (anti-PD-L1) and Ipilimumab (antiCTLA-4) for solid organ tumors. All these patients were followed in a single reference University Hospital from March-2015 up to December-2018. The main outcome was to determinate the incidence of irAEs. Results We studied 102 patients (63♂/39♀) with a mean age of 60.6±9.7 with lung (n=63), melanoma (21), kidney (11), gastric (3), bladder (1), and colon cancer (n=3). Only 7 patients had a previous diagnosis of an immune-mediated disease: psoriasis (n=2), psoriatic arthritis (1), systemic lupus erythematosus (1), spondyloarthitis (1), rheumatoid arthritis (1) and skin lupus (1). ICTB was performed as follows: nivolumab (n=52), pembrolizumab (35), atezolizumab (10) and ipilimumab (5). After a median of 5 [2.5-10.5] months since the ICBT onset, we observed 87 (85.3%) patients with different irAEs, summarized in TABLE. ICBT discontinuation was required in 39 patients. 36 patients received specific treatment (prednisone, antihistamine, levothyroxine and thiamazol), obtaining a good response in 31 cases (79.5%). ICBT was reintroduced in 28 patients (71.8%) after resolution of the adverse event, with an appropriate tolerance in all cases. Gastrointestinal irAEs were the most frequent (n=39, 41.1%), with severe manifestations in 4.9% of the patients. Among these 39 patients, only 11 of them required specific treatment, with oral prednisone in all cases. The diagnosis of gastrointestinal irAEs was based on the clinical manifestations and the laboratory tests. The cutaneous most frequent irAE was rash (n=7, 6.8%), followed by vitiligo (n=2, 1.9%). 15 patients developed a rheumatological irAE (14 arthralgias/arthritis and 1 aortitis). Patients who developed any type of irAE had a better answer to ICBT (70.1%) than those who did not (20%). Conclusion In our study, the majority of autoimmune side effects due to ICBT were gastrointestinal, thyroiditis and rheumatological. The develop of any irAE could be an indicator of good response to ICBT. Disclosure of Interests Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, Jose Luis Martin-Varillas: None declared, Marina Delgado Ruiz: None declared, Isabel Bernat Pina: None declared, Belen Atienza-Mateo: None declared, D. Prieto-Pena: None declared, Monica Calderon-Goercke: None declared, Almudena Garcia Castano: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi.

Background: Treatment of skin lupus lesions (SLL) was based on topical therapy (corticosteroids, calcineurin inhibitors), systemic corticosteroids, antimalarials and immunomodulatory drugs including thalidomide. However, there are refractory patients to conventional treatment. Apremilast, an inhibitor of phosphodiesterase-4 (PDE-4), may be useful in these cases. Objectives: Our objective was to assess the efficacy and safety of apremilast. Methods: We set up an observational study of 8 patients with diagnosis of refractory SLL and treatment with apremilast at a standard dose of 30 mg/ 12 hours. The outcome was the presence of improvement and the appearance of side effects. Results: 8 patients (7 female/1 male) with mean age of 39.9±10.6 years with SLL were studied. Patients were diagnosed as follows: discoid lupus (n=5) and systemic lupus erythematosus (SLE) (n=3). Patients with SLE were classified as lupus panniculitis (n= 1), polycyclic ring lupus (n=1) and psoriasiform lupus (n=1). All skin lesions were confirmed by biopsy (table). Before apremilast, all patients had received conventional therapy without improvement: topical corticosteroids (n=8), topical tacrolimus (n=4), oral corticosteroids (n=5), antimalarials (n=8), thalidomide (n=1), belimumab (n=3) and rituximab (n=2). The time between SLL diagnosis and apremilast beginning was 145.1±79.4 months. After a mean follow-up of 14.4±4.8 months, all patients presented improvement of skin lesions (4 of them with complete response). Due to the appearance of gastrointestinal symptoms (diarrhoea, vomiting) it was necessary to reduce the dose of apremilast to 30 mg/day in one patient and finally, it was discontinued due to no improvement with antiemetic and probiotic treatment. Conclusion: Apremilast seems useful and relatively safe in patients with refractory SLL to conventional treatment. Disclosure of Interests: Jose Luis Martin-Varillas: None declared, Belen Atienza-Mateo: None declared, J. Loricera: None declared, Susana Armesto: None declared, Eduardo Cuende: None declared, Juanjo J Alegre-Sancho: None declared, Clara Moriano: None declared, Vanesa Calvo-Rio: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Lara Sanchez Bilbao: None declared, Inigo Gonzalez-Mazon: None declared, C. Gonzalez-Vela: None declared, J. Luis Hernandez: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology which can lead to coronary aneurysm (CA) formation in 15-20% of patients. Long-term rates of adverse cardiac events are not well-known. Objectives: a) to compare demographic, clinical and laboratory features of patients who developed coronary aneurysm with patients who did not. b) to assess long-term cardiovascular outcomes in the subgroup of patients who developed cardiac aneurysms. Methods Single center study of 55 patients with KD who were diagnosed between 1994-2009. KD diagnosis was based on the classic classification criteria1. We considered two groups: a) patients who presented CA at diagnosis. b) patients who did not present CA. Statistical analysis was performed with SPSS. Student’s t test or Mann-Whitney U test was used to compare continuous variables, and Chi-squared test or Fisher’s exact test for categorical variables as appropriate. Results Out of 55 patients with KD, 8 patients (3 men/5 women) presented CA at diagnosis. Differences between the two groups mentioned above are shown in Table 1. Mean age at diagnosis was lower in patients who presented CA (1.74 vs 3.88 years; p=0.044). The duration of symptoms was longer in patients with CA (14.0 vs 12.0 days; p=0.015), however there were no qualitative differences in the symptomatology. Regarding laboratory markers, C- reactive protein (CRP) levels were significantly higher in patients who developed CA (17.0 vs 10.8 mg/dL; p=0.047). There was also a tendency to higher levels of erythrocyte sedimentation rate (ERS) and higher platelet count. All patients received intravenous immunoglobulin (IVIG) and antiplatelet agents at the time of diagnosis. Some of the patients with CA required glucocorticoids and anti-TNF agents. Table 2 shows the characteristics of the 8 patients with CA who were followed-up for a mean of 13.7 ± 7.8 years. The mean size of the aneurysms was 5.6 ± 2.3 mm. Left coronary artery was the most common location (n=5). In two patients, cardiac arrhytmia was described. Five patients underwent ergometry tests being negative for ischemia in all of them. Coronary tomography was performed in other two patients observing persistence of CA in just one of the patients after 131 months of follow-up. Conclusion In our cohort, patients with KD who presented CA at the time of diagnosis were younger, had a longer duration of symptoms and higher levels of CRP. All patients received early treatment with IVIG and antiplatelet agents. At long-term follow up, persistent coronary affection was reported in just one of the patients. No severe cardiovascular outcomes were shown. References [1] Wood LE, et al. Expert Rev Cardiovasc Ther. 2007; 5: 553-61 Disclosure of Interests D. Prieto-Pena: None declared, Jose Luis Martin-Varillas: None declared, Lara Sanchez Bilbao: None declared, Eva Pena Sainz-Pardo: None declared, Monica Calderon-Goercke: None declared, Natalia Palmou-Fontana: None declared, Maria Teresa Viadero: None declared, Maria Jesus Cabero: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background:Rheumatoid arthritis (RA) patients are at increased risk of severe infections due to the disease itself, and the immunosuppressive treatment. Vaccination programs are designed to decrease the risk of infections.Objectives:In patients with RA treated with biologic therapy (BT) our aim was to assess a) the incidence of severe respiratory infections and b) to compare the risk between vaccinated and non vaccinated patients.Methods:Observational study of 431 patients diagnosed with RA that iniciated BT. One group of patients participated in a vaccination program from October 2011 to October 2016 (Group 1). The other group was not included in the vaccination program (Group 2). The follow-up was made until June 2017 with a minimum follow-up period of 8 months and a maximum of 5.5 years.Information on severe respiratory infections, defined as those that required hospitalization or at least one dose of intravenous antibiotic treatment at the emergency room, was retrieved from the hospital medical records.Results:We studied 431 patients (335 women/96 men); mean age 63.4±13.7 years. In the vaccination program (group 1) were included 299 (69.37%) patients and in the group 2 132 patients (30.63%). The main features of both groups are summarized in Table 1.During the follow-up, we registered 299 hospital admissions due to severe respiratory infections in both groups (incidence density 9.9 (95% CI: 6.9-13.6).In group 1, vaccinated patients, this incidence density was reduced to 7.1 (95% CI: 4.1-11.6). Figure 1.The vaccination program reduced the general incidence of severe respiratory infection in 44%.Conclusion:RA patients with BT included in the vaccination program present a lower incidence of severe respiratory infections compared with non vaccinated patients.Table 1.Main general features at BT onsetGroup 1Vaccination programN=299Group 2Non vaccination programN=132pAge (years) mean±SD61.32±13.0467.97±14.170.32Women, n (%)231 (77.3%)105 (79.5%)0.59Duration of RA (months) mean±SD73.24±10.4112.62±60.2Positive RF/ Positive ACPA, n (%)177(59.2)/172 (57.52%)93(70.5%)98 (74.24%)0.02/0,01Erosive disease, n (%)116 (38.8%)70 (53%)0.06Vasculitis, n (%)15 (5%)2 (1.5%)0.08Interstitial lung disease n (%)12 (4%)7 (5.3%)0.54Subcutaneous nodules n (%)16 (5.4%)6 (4.5%)0.72Corticosteroids299 (100%)132 (100%)1Number of conventional DMARDs, mean±SD1.66±0.892.03±1.050.3Figure 1.Disclosure of Interests:None declared

Background:Hidradenitis suppurativa (HS) is a chronic inflammatory cutaneous disease affecting terminal hair follicles in apocrine glands bearing skin. The pathogenesis of HS remains unknown, although increasing evidence suggests that the immune system plays an important role. To date, two previous studies, did not find any association betweenHLAand HS.Objectives:Our aim was to analyze the association of HLA class II with HS in a Caucasian population from Cantabria (northern Spain).Methods:In this study we analyzed theHLA-A, -B, -C, DRB1, -DQA1 and –DQB1allele distribution in 106 HS patients and 262 age- and sex-matched controls from a Caucasian population of Cantabria (northern Spain).Results:HLA-A*29 andB*50 were significantly more frequent in HS patients andA*30 andB*37 in controls, but these associations disappeared after correction. On the other hand,DRB1*07,DQA1*02 andDQB1*02 were significantly more frequent in controls (p0.026,p0.0012 andp0.0005 respectively), and theHLAalleleDQB1*03:01 was significantly more frequent in HS patients (p0.00007) all of them after Bonferroni correction. Furthermore, theDRB1*07;DQA1*02;DQB1*02 haplotype was significantly more frequent in controls (p0.0005).Conclusion:This is the first study showing an association of HLA-class II with HS. Our results suggest that HLA-II alleles (DRB1*07,DQA1*02,DQB1*02 andDQB1*03:01) and theDRB1*07~DQA1*02~DQB1*02 haplotype could influence on resistance or susceptibility to HS.References:[1]González-López MA. J Am Acad Dermatol. 2016; Aug;75(2):329-35.[2]González-López MA. PLoS One. 2018 Jan 4;13(1).[3]Vilanova I. J Eur Acad Dermatol Venereol. 2018 May;32(5):820-824.[4]Durán-Vian C, et al. J Eur Acad Dermatol Venereol. 2019 Nov;33(11):2131-2136.Disclosure of Interests:Monica Calderón-Goercke: None declared, J. Gonzalo Ocejo-Vinyals: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Marcelo A. Fernández-Viña: None declared, Juan Cantos-Mansilla: None declared, Iosune Vilanova: None declared, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD, Marcos González-López: None declared

Background:18F-FDG PET/CT is useful to establish the presence and extent of large vessel vasculitis (LVV)(1-2).Early therapy is needed to prevent severe complications.Tocilizumab (TCZ) has shown efficacy in LVV(3-5).However, it is unknown if the extent of FDG vascular uptake may influence on clinical response to TCZObjectives:To assess the correlation of the extent of baseline FDG vascular uptake in PET/CT scan with clinical response to TCZ in patients with LVV.Methods:Single center study of patients with LVV treated with TCZ who were divided into 2 groups depending on the extent of vascular uptake in baseline PET/CT scan:a) 1-2 affected areasb) ≥affected 3 areas. Vascular uptake was qualitatively assessed by two experienced nuclear medicine physicians in five areas (supraaortic trunks, thoracic aorta, abdominal aorta, iliac and femorotibial arteries). We assessed clinical improvement (no improvement/partial/complete), normalization of CRP (≤0.5mg/dL) and/or ESR (≤ 20 mm/1sth) and reduction of prednisone dose (mg/day).Results:30 patients (24 w/6 m); mean age 65.3±10.6 yrs. In baseline PET/CT, vascular uptake was observed in 1 or 2 areas (n=13) and in ≥3 areas (n=17). There was a trend to higher ESR/CRP and shorter evolution of clinical symptoms before TCZ onset in patients with ≥3 affected areas (TABLE 1). Clinical/serological evolution and reduction of prednisone dose is shown in TABLE 2. No statistical differences were found. However, patients with ≥ 3 affected areas tended to experience a slower clinical response.TABLE 1.1-2 vascular affected areas (n=13)≥3 vascular affected areas (n=17)PDemographic dataAge,mean ± SD66.0 ± 10.864.8 ± 10.70.77Sex (women),n (%)11 (84.6)13 (76.5)0.67Evolution time before TCZ (months),median [IQR]26.0 [3.5-34.0]5.0 [1.5-10.0]0.02Laboratory markersESR (mm/1st h),mean ± SD30.0 ± 27.334.8 ± 27.60.64CRP (mg/dL),mean ± SD1.3 ± 1.21.8 ± 1.70.28Previous treatmentPrednisone dose (mg/day),mean ± SD9.4 ± 6.27.9 ± 6.90.53TCZ therapyIntravenous, n(%)10 (76.9)11 (64.7)0.47Combined with MTX, n(%)6 (46.2)8 (47.1)0.96TABLE 2.1-2 vascular affected areas (n=13)≥ 3 vascular affected areas (n=17)PComplete clinical improvement,n/N (%)6 m11/13 (84.6)12/17 (70.6)0.4312 m12/13 (92.3)13/17 (76.5)0.3618 m10/11 (90.9)11/12 (91.7)0.9924 m8/8 (100)9/10 (90.0)0.99Normalization of ESR and/or CRP,n/N (%)6 m13/13 (100)16/17 (94.1)0.9912 m13/13 (100)16/17 (94.1)0.9918 m11/11 (100)11/12 (91.7)0.9924 m8/8 (100)10/10 (100)0.99Dose of Prednisone (mg/day),median [IQR]6 m5.0 [1.3-5.0]5.0 [0.0-5.0]0.9812 m2.5 [0.0-3.8]0.0 [0.0-5.0]0.9718 m0.0 [0.0-2.5]0.0 [0.0-1.9]0.7224 m0.0 [0.0- 2.2]0.0 [0.0-2.5]0.77Conclusion:TCZ therapy was effective in patients with LVV regardless the extent of FDG vascular uptake in baseline PET/CT scan. However, a trend to a slower clinical response was observed in patients with ≥3 affected areas.References:[1]Martínez-Rodríguez et al. (18)F-FDG PET/CT in the follow-up of large-vessel vasculitis: A study of 37 consecutive patients. Semin Arthritis Rheum.2018 Feb;47(4):530-537. doi: 10.1016/j.semarthrit.2017.08.009.[2]Loricera et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015 Mar-Apr; 33(2 Suppl 89): S-19-31[3]Calderón-Goercke M et al. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Aug; 49(1):126-135. doi: 10.1016/j.semarthrit.2019.01.003[4]González-Gay MA et al. Current and emerging diagnosis tools and therapeutics for giant cell arteritis. Expert Rev Clin Immunol. 2018 Jul;14(7):593-605. doi: 10.1080/1744666X.2018.1485491.[5]Loricera et al. Tocilizumab in patients with Takayasu arteritis: a retrospective study and literature review. Clin Exp Rheumatol. 2016 May-Jun;34(3 Suppl 97): S44-53.Disclosure of Interests:D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Isabel Martínez-Rodríguez: None declared, Jose Ignacio Banzo: None declared, Patricia Vicente-Gómez: None declared, Javier García-Fernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, Roche, Consultant of: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Speakers bureau: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma. MSD

Background:Tocilizumab (TCZ) was recently approved for Takayasu Arteritis (TAK) in Japan based on the results of the TAKT trial(1).However, data in clinical practice in Europe and America are scarce(2).Objectives:To assess efficacy and safety of TCZ in TAK of clinical practice in Spain.Methods:Observational, open-label multicentre study of 53 TAK patients treated with TCZ due to refractoriness or adverse events of previous therapy. Outcomes variables were improvement of clinical features, acute phase reactants and glucocorticoid-sparing effect.Results:53 patients (46w/7m); mean age, 40.6±14.6 years at TCZ onset. TCZ was started after a median of 12 [3.0-48.0] months from TAK diagnosis. In addition to systemic corticosteroids and before TCZ they received conventional immunosuppressant drugs (n=42) and biologic therapy (n=14). TCZ was prescribed as standard I.V. (n=42; 79.2%) or subcutaneous (n=11; 20.8%). The initial dose was 8 mg/kg/IV/4 weeks or 162 mg/SC/week, respectively. TCZ was used in monotherapy or combined with immunosuppressants (n=32; 60.4%): methotrexate (n=27), azathioprine (n=2), cyclosporine (n=3). Main clinical features at TCZ onset were: malaise (n=30),limb claudication (n=22), headache (n=18), fever (n=14), abdominal pain (n=10), and chest pain (n=9). Most of the patients experienced a rapid and maintained clinical, analytical improvement(TABLE).After a median follow-up of 18.0 [7.0-45.0] months, TCZ was discontinued in 20 patients due to: sustained remission (n=6), relapse (n=6), adverse event (n=5), gestation (n=3). Most relevant adverse side effects were serious infections: pneumonia (n=2), herpes zoster (n=1), abdominal sepsis (n=1).Table.Basal(N=53)Month 1(N=53)Month 3(N=46)Month 6(N=44)Month 12(N=34)Clinical improvement, n/N(%)Complete17/53 (32.1)19/46 (41.3)23/44 (52.3)26/34 (76.5)Partial30/53 (54.6)26/46 (56.5)18/44 (40.9)8/34 (23.5)No improvement6/53 (11.3)1/46 (2.2)3/44 (6.8)0/34 (0.0)Analytical markers,ESR (mm/1sth),median [IQR]35.0 [16.0-52.0]7.5 [3.0-14.0] *3.5 [2.0-8.0]*5.0[2.0-6.0]*5.0 [2.0-8.5]*CRP (mg/dL),median [IQR]1.7 [0.6 -3.5]0.21 [0.05-0.6]*0.14 [0.05-0.5]*0.14 [0.04-0.4]*0.10 [0.03-0.30]*Hb (g/dL),mean±SD12.3±1.512.8±1.2*12.9±1.3*12.9±1.4*12.9±1.4*Prednisone dose (mg/day),median [IQR]30.0 [15.0-50.0]20.0 [10.0-37.5]*10.0 [5.0-20.0]*5.0 [5.0-12.5]*5.0 [0.0-7.5]**Wilcoxon test p < 0.001.Conclusion:TCZ appears to be effective and safe in patients with refractory TAK in clinical practice.References:[1]Nakaoka Y et al. Ann Rheum Dis. 2018;77:348-354[2]Loricera J et al. Clin Exp Rheumatol. 2016; 34: S44-53.Disclosure of Interests:D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Pilar Bernabéu: None declared, Paloma Vela-Casasempere: None declared, J. Narváez: None declared, Carlos Fernández-López: None declared, Mercedes Freire González: None declared, Beatriz González-Alvarez: None declared, Roser Solans-Laqué: None declared, Jose Luis Callejas-Rubio: None declared, Norberto Ortego: None declared, Carlos Fernández-Díaz Speakers bureau: Brystol Meyers Squibb, Esteban Rubio Romero: None declared, SALVADOR GARCÍA MORILLO: None declared, Mauricio Minguez: None declared, Cristina Fernández-Carballido Consultant of: Yes, I have received fees for scientific advice (Abbvie, Celgene, Janssen, Lilly and Novartis), Speakers bureau: Yes, I have received fees as a speaker (Abbvie, Celgene, Janssen, Lilly, MSD, Novartis), Eugenio de Miguel Grant/research support from: Yes (Abbvie, Novartis, Pfizer), Consultant of: Yes (Abbvie, Novartis, Pfizer), Paid instructor for: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Speakers bureau: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Sheila Melchor: None declared, Eva Salgado-Pérez: None declared, Beatriz Bravo: None declared, Susana Romero-Yuste: None declared, J Salvatierra: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, C. Romero-Gómez: None declared, Patricia Moya: None declared, Noelia Alvarez-Rivas: None declared, Javier Mendizabal: None declared, Francisco Miguel Ortiz Sanjuan: None declared, I. Pérez de Pedro: None declared, Javier Loricera: None declared, Santos Castañeda: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, Roche, Consultant of: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Speakers bureau: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma. MSD

Background:Anti-TNFα agents are useful in uveitis(1-5).Certolizumab pegol (CZP) differs from other anti-TNFα agents due to its limited placental transfer.Objectives:To assess efficacy and safety of CZP in women with uveitis during pregnancy.Methods:Multicenter study of women with uveitis under CZP during pregnancy and their neonates.Results:14 women (23 eyes); mean age 34.3±5.5 yrs (TABLE 1). Pattern of uveitis: 10 anterior, 2 posterior, 1 intermediate, 1 panuveitis. Uveitis was bilateral in 9 and chronic in 7. CZP was started before conception in 10 patients and after in 4. All patients obtained or maintained ocular remission throughout pregnancy (FIGURE). Prednisone was reduced from a mean dose of 21.7±19.7 mg/day to 4.1±3.8 mg/day at 6 months (p=0.03), leading to complete discontinuation in 4. 15 healthy infants were born. Only 1 woman presented a mild infection. No infections or malformations were found in neonates after a follow-up of 6 months. 6 infants were breastfed and all received scheduled vaccinations without complications (TABLE 2).TABLE 1.AgeUnderlying diseaseImmunosuppressants before CZPCombined treatment134SpAMTX, AZA, ADAAZA237SpAMTX, AZA, IFX, ADA, GOLI339SpAAZA, ADAAZA446SpACyA, ETN, ADA, IFX, GOLI532SpASSZ, ADASSZ636SpAMTX, HCQ, ADA740SpAMTX, LFN, HCQ, IFX, ADA, GOLIHCQ831IdiopathicMTX, MMF, CyA, ADA933IdiopathicMTX, AZA, ADA, ETN1032RAMTXAZA1123Vogt-Koyanagi-HaradaAZA, ADAAZA1236Juvenil Idiopathic ArthritisADA1332Punctate inner choroidopathyADA1429BehcetCyA, IFX, ADAConclusion:CZP seems to be effective and safe in female patients with uveitis during pregnancy and neonates.References:[1]Llorenç V et al. Certolizumab Pegol, a New Anti-TNF-α in the Armamentarium against Ocular Inflammation. Ocul Immunol Inflamm. 2016;24(2):167-72. doi: 10.3109/09273948.2014.967779[2]Urruticoechea-Arana A et al. Efficacy and safety of biological therapy compared to synthetic immunomodulatory drugs or placebo in the treatment of Behçet’s disease associated uveitis: a systematic review. Rheumatol Int. 2019 Jan;39(1):47-58. doi: 10.1007/s00296-018-4193-z[3]Martín-Varillas JL et al. Successful Optimization of Adalimumab Therapy in Refractory Uveitis Due to Behçet’s Disease Ophthalmology. 2018 Sep;125(9):1444-1451. doi: 10.1016/j.ophtha.2018.02.020[4]Santos-Gómez M et al. The effect of biologic therapy different from infliximab or adalimumab in patients with refractory uveitis due to Behçet’s disease: results of a multicentre open-label study. Clin Exp Rheumatol. 2016. Sep-Oct;34(6 Suppl 102): S34-S40[5]Calvo-Río V et al. Golimumab in refractory uveitis related to spondyloarthritis. Multicenter study of 15 patients.Semin Arthritis Rheum. 2016 Aug;46(1):95-101. doi: 10.1016/j.semarthrit.2016.03.002Disclosure of Interests:D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Alfredo Adan: None declared, Lillian Chamorro-López: None declared, Olga Maiz: None declared, JR De Dios-Jiménez Aberásturi: None declared, Raul Veroz Gonzalez: None declared, Soledad Blanco: None declared, José M Santos: None declared, Francisco Navarro: None declared, Adela Gallego: None declared, Senen González-Suárez: None declared, Arantxa Conesa: None declared, Andrea García-Valle: None declared, Miguel Cordero-Coma: None declared, Nieves Pardiñas-Barón: None declared, Rosalía Demetrio-Pablo: None declared, Vanesa Calvo-Río Grant/research support from: MSD and Roche, Speakers bureau: AbbVie, Lilly, Celgene, Grünenthal, UCB Pharma, Victor Manuel Mora-Cuesta: None declared, Santos Castañeda: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, Roche, Consultant of: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Speakers bureau: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma. MSD

Background:The eye is a common and potential severe complication of sarcoidosis. Topical and systemic corticosteroids are the first-line treatment. Conventional and biological immunosuppressants (IS) are frequently needed(1-5).Objectives:To assess the frequency, clinical and treatment of ocular involvement of sarcoidosis.Methods:Study of a large cohort (n=383) of systemic sarcoidosis from a single university hospital. All consecutive patients diagnosed with sarcoidosis from January 1,1999 to January 1,2019 according the ATS/ERS/WASOG criteria(Eur Respir J 1999;14:735–737) were included.Results:41 (22 women/19 men) of 383 (10.7%) patients had ocular involvement, mean age 44.8±16 years. Uveitis (n=34; 82.9%) was the most common ocular manifestation, especially anterior uveitis (n=18; 52.9%). Ocular surface and eye orbit may also be affected(Table). In addition to topical and systemic corticosteroids, conventional (n=23; 56.1%) and biologic (n=14; 34.1%) IS drugs were required. Adalimumab and Infliximab were the most used biologic treatments(Table).Cystoid macular edema (CME) and Retinal Vasculitis was observed in both cases in 3 (7.3%) patients, 2 of them (66.7%) required biological treatment. Papilitis appeared in 7 (17.1%) cases, biological treatment was needed in 3 (42.9%) patients. The most frequent sequels were cataract (n=9, 21.9%), intraocular hypertension (n=5; 12.2%) and pupil alterations (n=4; 9.7%). The average of the best corrected visual acuity was 0.6±0.3 at diagnosis and 0.7±0.3 after one year follow up.Conclusion:Ocular involvement of sarcoidosis is a relative frequent and potential severe complication, especially if panuveitis is presented.References:[1]Riancho-Zarrabeitia L, et al. Semin Arthritis Rheum. 2015; 45:361-8.[2]Calvo-Río V, et al. Clin Exp Rheumatol. 2014; 32:864-8.[3]Riancho-Zarrabeitia L, et al. Clin Exp Rheumatol. 2014; 32:275-84.[4]Vegas-Revenga N, et al. Am J Ophthalmol. 2019; 200:85-94.[5]Calvo-Río V, et al. Clin Exp Rheumatol. 2014;32(4 Suppl 84): S54-7.Table.Ocular manifestations of sarcoidosis and treatment with corticosteroids, conventional and biological IS.CONVENTIONAL ISBIOLOGICAL ISOCULAR INVOLVEMENTCasesTCSOCSMD of OCSIVMPCISMTXAZACFMMMFBTADAIFXTCZGLMETNSURFACE n(%)3(7.3)2(66.7)2(66.7)2(66.7)2(66.7)2(66.7)2(66.7)1(33.3)0(0)2(66.7)2(66.7)2(66.7)0(0)0(0)0(0)-CG/N, n(%)1(33.3)1(100)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)-PUK, n(%)2(66.7)1(50)2(100)602(100)2(100)2(100)2(100)1(50)0(0)2(100)2(100)2(100)0(0)0(0)0(0)UVEITIS n(%)34(82.9)25(73.5)28(82.3)10(29.4)19(55.6)18(52.9)7(20.6)1(2.9)1(2.9)12(35.3)11(32.3)4(11.8)3(8.8)2(5.9)1(2.9)-Anterior uveítis, n(%)18(52.9)11(61.1)13(72.2)301(5.5)5(27.8)5(27.8)1(5.5)0(0)0(0)0(0)0(0)0(0)0(0)0(0)0(0)-Posterior uveítis, n(%)4(11.7)2(50)3(75)601(25)3(75)2(50)2(50)0(0)0(0)2(50)1(25)0(0)0(0)1(25)0(0)-Panuveítis, n(%)12(35.3)12(100)12(100)608(66.7)11(91.7)11(91.7)4(33.3)1(8.3)1(8.3)10(83.3)10(83.3)4(33.3)3(25)1(8.3)1(8.3)EYE ORBIT n(%)4(9.7)2(50)3(75)2(50)2(50)2(50)2(50)0(0)0(0)2(50)1(25)1(25)1(25)0(0)0(0)-Proptosis, n(%)2(50)1(50)1(50)301(50)1(50)1(50)1(50)0(0)0(0)1(50)1(50)0(0)1(50)0(0)0(0)-Strabismus, n(%)2(50)1(50)2(100)601(50)1(50)1(50)1(50)0(0)0(0)1(33.3)0(0)1(33.3)0(0)0(0)0(0)TOTAL, n(%)41(100)29(70.7)33(80.5)50±15.514(34.1)23(56.1)22(53.7)11(26.9)2(4.9)1(2.4)14(34.1)14(34.1)7(17.5)3(7.3)2(4.9)1(2.4)TCS:topical corticosteroids;OCS:oral corticosteroids;MD:maximum dose;IVMP:intravenous methylprednisolone;CIS: conventional immunossupresors;BT:biologic therapy;CG/N:conjunctival granuloma/nodule;PUK:peripheral ulcerative keratitisDisclosure of Interests:Monica Calderón-Goercke: None declared, Jorge Javier Gaitán-Valdizán: None declared, Raúl Fernández-Ramón: None declared, Lara Sánchez-Bilbao: None declared, Rosalía Demetrio-Pablo: None declared, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Background:Infections are the most common adverse event of Tocilizumab (TCZ) in Giant Cell Arteritis (GCA). In GiACTA study(1),serious infections were observed in 7% (9.6/100 patient-years) of patients who received TCZ weekly. Randomized clinical trials (RCTs) are conducted under highly standardized design excluding some real-world patients. Therefore, adverse events may be underestimated in RCTs. In our series of real-life, serious infections occurred in 11.9% (10.6/100 patient-years)(2).Objectives:In a wide series of GCA of clinical practice treated with TCZ, we assess the frequency, type and predisposing factors of serious infections.Methods:Multicenter study of 134 patients diagnosed with GCA, all of them refractory to conventional therapy, treated with TCZ. Serious infection was considered when a life-threatening infection, fatal, or requiring hospitalization occurred, intravenous antibiotics were required, or the infectious process led to persistent or significant disability.Results:16 of 134 (11.9%, 10.6/100 patient-years) patients developed serious infections during follow-up. The most frequent infections were pneumonia (n=4), urinary tract infection (n=4), and facial herpes zoster (n=2). At TCZ onset, serious infections were more frequent in older patients (74.3±9.6 vs 72.9±8.7 years), with a longer GCA evolution (20 [4.3-45.6] vs 13 [5-29.3] months), with visual manifestations (43.75% vs 17.8%) and a higher dose of prednisone at TCZ onset (30.4±15.5 vs 21.1±16.1 mg/day) (TABLE). Presence of comorbidities were similar in both groups. 13 of the 16 patients who had infections received a dose of prednisone greater than 15 mg/day (16.3/100 patient-years) compared to 3 patients under treatment with less than 15 mg/day of prednisone (4.2/100 patient-years).Conclusion:The age, GCA duration, ocular involvement and the dose of glucocorticoids, at TCZ onset, seem to be predisposing factors related to an increased risk of developing serious infections in GCA patients.References:[1]Stone JH, et al. N Engl J Med. 2017; 377:317-28.[2]Calderón-Goercke M et al. Semin Arthritis Rheum 2019 Aug;49(1): 126-135.TABLESERIOUS INFECTIONS(n=16)WITHOUT SERIOUS INFECTIONS(n=118)pBASAL FEATURES AT TCZ ONSETGENERAL FEATURES Age, years, mean± SD74.3±9.672.9±8.70.552 Sex, female/male n(%)13/388/300.760 Time from GCA diagnosis to TCZ onset (months), median [IQR]20[4.3-45.6]13[5-29.3]0.604COMORBIDITIES Hypertension, n(%)9(56)86(73)0.551 Diabetes, n(%)3(19)39(33)0.677 Chronic kidney disease, n(%)3(19)27(23)0.512CLINICAL FEATURES OF GCA PMR, n(%)9(56.25)64(54.2)0.879 Aortitis, n(%)5(31.25)53(45)0.301 Visual manifestations, n(%)7(43.75)21(17.8)0.017CORTICOSTEROIDS AT TCZ ONSET Prednisone dose mg/d, mean (SD)30.4±15.521.1±16.10.031Disclosure of Interests:Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Santos Castañeda: None declared, Clara Moriano: None declared, Elena Becerra-Fernández: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Águeda Prior-Español: None declared, E. Galindez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eugenio de Miguel Grant/research support from: Yes (Abbvie, Novartis, Pfizer), Consultant of: Yes (Abbvie, Novartis, Pfizer), Paid instructor for: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Speakers bureau: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Eva Salgado-Pérez: None declared, Vicente Aldasoro Speakers bureau: Roche, Abbvie, MSD, UCB, Pfizer, Menarini, Grunenthal, Gebro, Novartis, Janssen, Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Susana Romero-Yuste: None declared, J. Narváez: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampín: None declared, Rafael Melero: None declared, Francisca Sivera: None declared, Carlos Fernández-Díaz Speakers bureau: Brystol Meyers Squibb, Alejandro Olive: None declared, María Álvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernández-López: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Beatriz Arca: None declared, Roser Solans-Laqué: None declared, Arantxa Conesa: None declared, Carlos Vázquez: None declared, Jose Andrés Román-Ivorra: None declared, Pau Lluch: None declared, Paloma Vela-Casasempere: None declared, Carmen Torres-Martín: None declared, Juan Carlos Nieto Speakers bureau: Pfizer, Abbvie, MSD, Novartis, Janssen, Lilly, Nordic Pharma, BMS, Gebro, FAES Farma, Roche, Sanofi, Carmen Ordas-Calvo: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Nagore Fernández-Llanio: None declared, Antonio García: None declared, Carmen González-Vela: None declared, Javier García-Fernández: None declared, Patricia Vicente-Gómez: None declared, Ángel García-Manzanares: None declared, Norberto Ortego: None declared, Francisco Ortiz-Sanjuán: None declared, Montserrat Corteguera: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Background:Tocilizumab (TCZ) is the only biological agent approved in Giant Cell Arteritis (GCA). There is general agreement on the initial and the standard maintenance dose of TCZ. However, information on duration and optimization of TCZ in GCA is scarce.Objectives:Our aim was to assess efficacy and safety of TCZ therapy optimization in an unselected wide series of GCA in clinical practice.Methods:Multicenter study, 134 patients with GCA who received TCZ due to inefficacy/adverse events of previous therapy. Once complete remission was reached and based on a shared decision between patient and physician TCZ was optimized in some cases. Optimization was done by decreasing the dose and/or prolonging the TCZ dosing interval progressively.Results:134 GCA patients treated with TCZ (101w/33m); mean age 73.0±8.8 years. TCZ was administered IV to 106 (79.1%) patients and SC to 28 (20.9%). TCZ was optimized in 43 (32.1%) patients. No demographic, clinical manifestations or laboratory data differences had been found at TCZ onset (TABLE). After a follow up of 12 [6-15.5] months, and a complete remission for 6 [3-12] months; the first TCZ optimization was performed. Median prednisone dose at first TCZ optimization was 2.5 [0-5] mg/day. TCZ IV was optimized from 8 to 4 mg/kg/4weeks in 12 of 106 (11.3%) and from 162 mg/SC/week to 162 mg/SC/2weeks in 9 of 28 (32.1%) cases. Five (11.6%) of the 43 optimized cases relapsed. In 4 cases, the relapses were treated increasing TCZ up to the pre-optimization dose, in 1 case the route of administration was change (4 mg/kg/4week to 162 mg/SC/week). In 8 of 43 optimized patients (18.6%), it was possible to withdraw TCZ after complete remission for 30 [16.25-45.75] months. Regarding adverse events and severe infections were similar in both groups. The mean TCZ treatment costs were lower in the optimized group.Conclusion:Once remission is reached in GCA patients under TCZ treatment, optimization of TCZ may be performed. Based on our experience it could be performed by reducing the dose with IV TCZ or by prolonging dosing interval with SC TCZ.References:[1]Calderón-Goercke M et al. Semin Arthritis Rheum 2019 Aug;49(1): 126-135.TABLE.OPTIMIZED-TCZ GROUP (n=43)NON-OPTIMIZED TCZ GROUP (n=91)pBASAL FEATURES AT TCZ ONSETGENERAL FEATURESAge, years, mean± SD68.9±8.771.4±8.50.125Sex, female/male n(%)32/1068/240.779Time from GCA diagnosis to TCZ onset (months), median [IQR]19.5[7.75-45]10.5[4 – 25]0.047SYSTEMIC MANIFESTATIONSFever, n(%)1(2.4)8(8.7)0.176Constitutional syndrome, n(%)11(26.2)19(20.7)0.476PMR, n(%)18(42.9)56(60.9)0.052ISCHEMIC MANIFESTATIONSVisual involvement, n(%)5(11.9)23(25)0.084Headache, n(%)26(61.9)42(45.7)0.081Jaw claudication, n(%)1(2.4)11(12)0.072CORTICOSTEROIDS AT TCZ ONSETPrednisone dose, mg/d mean (SD)15.1±11.125±17.40.001FOLLOW-UP ON TCZ THERAPY (MONTHS), MEDIAN [IQR]24[18-27]6 [3-18]0.000Relapses, n(%)5(11.6)5(5.5)0.207End follow-up remission, n(%)40(93)84(92)0.99Severe side efects, n(%)14(32.6)22(24.2)0.307Seriuos infections, n(%)6(14)10(11)0.878Cost, (mean) euros per yearIVSC7 538.47 329.011 726.411 726.4--Disclosure of Interests:Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Santos Castañeda: None declared, Clara Moriano: None declared, Elena Becerra-Fernández: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Águeda Prior-Español: None declared, E. Galindez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eugenio de Miguel Grant/research support from: Yes (Abbvie, Novartis, Pfizer), Consultant of: Yes (Abbvie, Novartis, Pfizer), Paid instructor for: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Speakers bureau: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Eva Salgado-Pérez: None declared, Vicente Aldasoro Speakers bureau: Roche, Abbvie, MSD, UCB, Pfizer, Menarini, Grunenthal, Gebro, Novartis, Janssen, Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Susana Romero-Yuste: None declared, J. Narváez: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampín: None declared, Rafael Melero: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, María Álvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernández-López: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Beatriz Arca: None declared, Roser Solans-Laqué: None declared, Arantxa Conesa: None declared, Carlos Vázquez: None declared, Jose Andrés Román-Ivorra: None declared, Pau Lluch: None declared, Paloma Vela-Casasempere: None declared, Carmen Torres-Martín: None declared, Juan Carlos Nieto Speakers bureau: Pfizer, Abbvie, MSD, Novartis, Janssen, Lilly, Nordic Pharma, BMS, Gebro, FAES Farma, Roche, Sanofi, Carmen Ordas-Calvo: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Nagore Fernández-Llanio: None declared, Antonio García: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Background:Tocilizumab (TCZ) has shown efficacy in large vessel vasculitis (LVV)(1-3). Disease activity assessed by laboratory markers (ESR,CRP) may be of less value with TCZ.18F-FDG PET/CT may be useful to monitor LVV disease activity(4-5).Objectives:To assessa) evolution of disease activity in LVV treated with TCZ by PET/CT andb) its correlation with clinical/serological markers.Methods:Single centre study of 30 patients with refractory LVV treated with TCZ who had a baseline and follow-up PET/CT scan. Vascular uptake was assessed quantitatively and qualitatively. Quantitative analysis was assessed as a target to background ratio (TBR)=SUVmax thoracic aorta/SUVmax aortic vascular pool. For qualitative analysis, FDG uptake at vessel wall was visually grading compared to the liver. We defined a total vascular score which included 5 vascular areas (supra aortic trunks, thoracic, abdominal, iliac and femorotibial arteries) ranging from 0 to 15. Clinical improvement (no improvement/partial/complete), analytical (CRP mg/dL; ESR mm/1sthour) and reduction of prednisone dose (mg/day) were also assessed.Results:30 patients (24 w/6 m); mean age 65.3 ± 10.6 yrs. TCZ was started after 6.5 [2.0-20.0] months from LVV diagnosis. Most patients received TCZ as intravenous infusions (70%) and almost half of them (46.7%) received combined therapy with MTX. Clinical/analytical evolution and quantitative/qualitative uptake assessment is shown inTABLE. After a mean follow-up of 37.0±18.5 months, 92.3% of patients experienced complete clinical/analytical improvement. Complete quantitative normalization of vascular uptake (TBR< 1.34) was achieved in 30.8%. Qualitatively, 23.1% of patients showed normalization (total vascular score =0) at the end of the study period.Conclusion:Most patients with LVV under TCZ experienced rapid and effective clinical and analytical response. Decrease of vascular uptake was also observed both quantitatively and qualitatively assessed. However, complete normalization of vascular uptake despite clinical remision was only observed in less than one-third of patients.TABLE.Basal(n=30)6 months(n=9)12-18 months(n=21)>18 months(n=13)Clinical improvementComplete,n (%)7 (77.8)16 (76.2)12 (92.3)Laboratory markersESR (mm/1sth),median [IQR]24.0 [9.8-53.0]2.0 [2.0-3.0] *2.0 [2.0-4.0] *2.0[2.0-3.5] *CRP (mg/dL),median [IQR]1.5 [0.5-2.4]0.1 [0.1-0.2] *0.1 [0.1-0.1] *0.1 [0.1-0.1] *ESR/CRP normalization, n(%)9 (100)21 (100)13 (100)FDG vascular uptakeTBR,mean ± SD1.69 ± 0.521.56±0.41 *1.46±0.16 *1.40 ± 0.18 *Total vascular score,mean ± SD5.0 ± 2.63.7 ± 2.23.3 ± 1.7*2.7 ± 2.4*Quantitative normalization, n(%)4 (44.4)5 (23.8)4 (30.8)Qualitative normalization, n(%)1 (11.1)1 (4.8)3 (23.1)*test Wilcoxon: p < 0.05. Quantitative normalization when TBR References:[1]Calderón-Goercke M et al. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Aug; 49(1):126-135. doi: 10.1016/j.semarthrit.2019.01.003.[2]Loricera et al. Tocilizumab in patients with Takayasu arteritis: a retrospective study and literature review. Clin Exp Rheumatol. 2016 May-Jun;34(3 Suppl 97): S44-53.[3]González-Gay MA et al. Current and emerging diagnosis tools and therapeutics for giant cell arteritis. Expert Rev Clin Immunol. 2018 Jul;14(7):593-605. doi: 10.1080/1744666X.2018.1485491.[4]Martínez-Rodríguez et al. (18)F-FDG PET/CT in the follow-up of large-vessel vasculitis: A study of 37 consecutive patients. Semin Arthritis Rheum.2018 Feb;47(4):530-537. doi: 10.1016/j.semarthrit.2017.08.009.[5]Loricera et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015 Mar-Apr; 33(2 Suppl 89): S-19-31.Disclosure of Interests:D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Isabel Martínez-Rodríguez: None declared, Jose Ignacio Banzo: None declared, Javier García-Fernández: None declared, Patricia Vicente-Gómez: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, Roche, Consultant of: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Speakers bureau: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma. MSD

Background:Hidradenitis suppurativa (HS) is a chronic, relapsing inflammatory cutaneous disease affecting terminal hair follicles in apocrine-gland bearing skin. The pathogenesis of HS is still unknown, although increasing evidence suggests that the immune system plays an important role. In order to study the role of innate immunity we analyzed several Toll Like Receptors (TLRs) functional single nucleotide polymorphisms (SNPs). To date, only one previous study focused about the role of TLR4 SNPs in HS showing no association with this disease.Objectives:The main goal of this study was to analyze the role of several TLRs functional SNPs in HS patients and healthy controls, in a Caucasian population from Cantabria (northern Spain).Methods:Through a case-control study, we analyzed the allele and genotype distribution of the SNPs in 106 patients with HS and 278 age and sex matched healthy control subjects for the following SNPs (TLR1 rs5743611 and rs4833095, TLR2 rs5743704 and rs5743708, TLR6 rs5743810, and TLR10 rs11096955, rs11096957 and rs4129009, by Real-Time PCR using a TaqMan assay.Results:We did not find any significant difference in the allelic distribution of the different SNPs between HS patients and controls. Regarding genotypes, only TLR10 rs11096955 (dominant, codominant and overdominant), rs11096957 (dominant, codominant and overdominant) and rs4129009 (codominant and overdominant) showed significant differences between HS patients and controls. However, no association was found when we analyzed the different TLR10 haplotypes.Conclusion:To the best of our knowledge, this is the first study showing an association of TLR10 SNPs with HS.References:[1]González-López MA. J Am Acad Dermatol. 2016; Aug;75(2):329-35.[2]González-López MA. PLoS One. 2018 Jan 4;13(1)[3]Vilanova I. J Eur Acad Dermatol Venereol. 2018 May;32(5):820-824.[4]Durán-Vian C, J Eur Acad Dermatol Venereol. 2019 Nov;33(11):2131-2136.Disclosure of Interests:Monica Calderón-Goercke: None declared, J. Gonzalo Ocejo-Vinyals: None declared, Juan Irure-Ventura: None declared, María Gutiérrez-Larrañaga: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Iosune Vilanova: None declared, Juan Cantos-Mansilla: None declared, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD, Marcos González-López: None declared

Background:Sarcoidosis is a multisystemic disease characterized by the formation of non-necrotizing granulomas. The most frequently affected organs are lungs, skin and eyes. Systemic corticosteroids are the most used drugs in the treatment of this disease. Conventional and biological immunosuppressants (IS) may also be used(1-3).Objectives:To evaluate the systemic treatment of sarcoidosis according to clinical domains.Methods:Study of all consecutive patients diagnosed with sarcoidosis between 1/1/1999 and 1/1/2019 in a tertiary university hospital. The diagnosis was established following the ATS/ERS/WASOG criteria(Eur Respir J. 1999;14(4):735-737):compatible clinical and radiological presentation, evidence of non-caseifying granulomas and exclusion of other granulomatous diseases.Results:We studied 377 patients (188 men/189 women), mean age at diagnosis of 46.0±14.8 years. After a mean follow-up of 13.0±9.3 years, 161 (42.7%) patients did not require treatment. The remaining 216 (57.3%) received oral glucocorticoids (206, 54.6%) with a maximum mean dose of 43.2±19.0 mg/day, conventional IS (60, 16.2%), biological therapy (28, 7.4%) and/or endovenous methylprednisolone (15, 4.0%). Biological therapy was indicated by pulmonary (9, 32.1%), ocular (9, 32.1%), neurological (3, 10.7%), muskuloeskeletal (3, 10.7%), cutaneous (2, 7.1%), nephrological involvement (1, 3.6%); and Heerfordt’s syndrome (1, 3.6%). Adalimumab and Infliximab were the biologics used more frequently (Table).TABLESystemic treatment of sarcoidosis according to clinical domains.Conclusion:Compared to other studies, the high percentage of patients who required systemic treatment is remarkable. It also highlights the frequency of the use of biological drugs in more severe organ involvement (ocular and neurological), which is consistent with the trend in recent years.References:[1]Riancho-Zarrabeitia L, et al. Semin Arthritis Rheum. 2015; 45:361-8.[2]Vegas-Revenga N, et al. Am J Ophthalmol. 2019; 200:85-94.[3]Calvo-Río V, et al.. Clin Exp Rheumatol. 2014;32(4 Suppl 84): S54-7.Disclosure of Interests:Monica Calderón-Goercke: None declared, Raúl Fernández-Ramón: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Lara Sanchez-Bilbao Grant/research support from: Pfizer, David Martínez-López: None declared, Iñigo González-Mazón: None declared, Jorge Javier Gaitán-Valdizán: None declared, Rosalía Demetrio-Pablo: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Giant cell arteritis (GCA) is the most common form of vasculitis in adults. Cranial manifestations are typical clinical features of this vasculitis. Sometimes the presenting symptoms are nonspecific and, in some cases, large-vessel involvement may prevail. Polymyalgia rheumatica is a frequent manifestation that in some cases may be the presenting symptom of GCA. Visual complications, in particular the risk of blindness, constitute the most feared manifestations of GCA. Prompt recognition of this vasculitis is required to avoid irreversible complications. Prednisone/prednisolone at a dose of 40–60 mg/day is the cornerstone therapy in GCA. Glucocorticoids lead to rapid improvement of symptoms and may reduce the risk of irreversible visual loss. However, relapses are common when the prednisone dose is tapered. Therefore, additional therapies are required in relapsing GCA or when a rapid reduction of glucocorticoids is needed. The most widely used conventional immunosuppressive drug is methotrexate Adjunctive treatment with methotrexate may decrease the risk of relapses and reduce glucocorticoid exposure. However, comprehensive reviews indicate that the efficacy of methotrexate in GCA is modest. The experience with other conventional immunosuppressive drugs in GCA patients is scarce. In some cases, the new biologic agents are required. Among them, the most frequently used is the recombinant humanized anti-IL-6 receptor antibody tocilizumab. It improves clinical symptoms, reduce the cumulative prednisone dose and the frequency of relapses in GCA patients. However, anti-tumor necrosis factor-α therapy is not useful in GCA. Promising results on other biologic agents, such as abatacept, ustekinumab or anakinra, require further confirmatory studies.

Objective: Tocilizumab (TCZ) has shown efficacy in clinical trials on giant cell arteritis (GCA). Real-world data are scarce. Our objective was to assess efficacy and safety of TCZ in unselected patients with GCA in clinical practice Methods: Observational, open-label multicenter study from 40 national referral centers of GCA patients treated with TCZ due to inefficacy or adverse events of previous therapy. Outcomes variables were improvement of clinical features, acute phase reactants, glucocorticoid-sparing effect, prolonged remission and relapses. A comparative study was performed: (a) TCZ route (SC vs. IV); (b) GCA duration (6 months); (c) serious infections (with or without); (d) < 15 vs. >15 mg/day at TCZ onset. Results: 134 patients; mean age, 73.0 +/- 8.8 years. TCZ was started after a median [IQR] time from GCA diagnosis of 13.5 [5.0-33.5] months. Ninety-eight (73.1%) patients had received immunosuppressive agents. After 1 month of TCZ 93.9% experienced clinical improvement. Reduction of CRP from 1.7 [0.4-3.2] to 0.11 [0.05-0.5] mg/dL (p < 0.0001), ESR from 33 [14.5-61] to 6 [2-12] mm/1st hour (p < 0.0001) and decrease in patients with anemia from 16.4% to 3.8% (p < 0.0001) were observed. Regardless of administration route or disease duration, clinical improvement leading to remission at 6, 12, 18, 24 months was observed in 55.5%, 70.4%, 69.2% and 90% of patients. Most relevant adverse side-effect was serious infections (10.6/100 patients-year), associated with higher doses of prednisone during the first three months of therapy. Conclusion: In clinical practice, TCZ yields a rapid and maintained improvement of refractory GCA. Serious infections appear to be higher than in clinical trials. (C) 2019 Elsevier Inc. All rights reserved.

Introduction: Giant cell arteritis is a vasculitis of large and middle-sized arteries that affects individuals older than 50 years. Although glucocorticoids remain the mainstay in the treatment of this vasculitis, other drugs are often required to achieve clinical remission and allow glucocorticoid discontinuation. Areas covered: The review summarizes the main biologic therapies used for the managements of GCA. Expert commentary: Although several biologic agents have been used in patients with GCA, the only biologic agent currently approved for this purpose is the recombinant humanized anti-IL-6 receptor antibody: tocilizumab. It has demonstrated efficacy to improve clinical symptoms, decrease the cumulative prednisone dose and reduce the frequency of relapses in clinical trials and real-life studies on patients with GCA. A trial showed that abatacept may be useful to maintain remission in GCA patients. An openlabel study suggested that ustekinumab could be useful for the treatment of patients with refractory GCA. However, further studies are required to confirm if both abatacept and ustekinumab are useful as an adjunctive therapy to reduce relapses or as a glucocorticoid sparing agent in GCA. Anakinra has been successfully used in a few patients with refractory GCA. In contrast, anti-tumor necrosis factor- α therapy yielded disappointing results in GCA.

Introduction: Giant cell arteritis (GCA) is the most common large-vessel vasculitis in individuals older than 50 years from Western countries. The goal of the treatment is to achieve improvement of symptoms and clinical remission as well as decrease the risk of severe vascular complications. Areas covered: The review summarizes the main epidemiological and clinical features of GCA and discusses in depth both the classic and the new therapies used in the management of GCA. Expert commentary: Prednisone/prednisolone of 40-60 mg/day is the mainstay in GCA therapy. It yields improvement of clinical features and reduces the risk of permanent visual loss in patients with GCA. Other drugs are used in patients who experience relapses (flares of the disease) or side effects related to glucocorticoids. Methotrexate is the most common conventional immunosuppressive drug used as a glucocorticoid sparing agent. Among the new biologic agents, the most frequently used is the recombinant humanized anti-IL-6 receptor antibody, which is effective to improve clinical symptoms, decrease the cumulative prednisone dose and reduce the frequency of relapses in these patients. Anti-tumor necrosis factor-α therapy is not useful in GCA. Experience with other biologic agents, such as abatacept or ustekinumab, looks promising but it is still scarce.

Background Giant cell arteritis (GCA) is a large vessel vasculitis with a predisposition for the cranial branches of the external carotid artery. However, aorta and/or its main branches may also be involved (1–3. Objectives To assess the vascular territories most frequently affected in a series of patients with GCA who presented extracranial vessel involvement. Methods Retrospective study of patients with GCA who presented compromise of extracranial vessels confirmed by PET/CT. Visual analysis of vascular uptake was performed on supra-aortic trunks (SAT), aortic arch (AA), thoracic aorta (TA), abdominal aorta (AA), iliac arteries (IA), lower limb arteries (LLA), and upper limb arteries (ULA). We carried out a comparative study between both sexes to see if there was any difference in the pattern of affectation. Results We evaluated 68 patients with GCA (51W/17M) with a mean age of 68.06±8.33 years. The vascular territories affected were: TA (n=58, 85.29%), SAT (n=38, 55.88%), AA (n=28, 41.18%), AA (n=18, 26.47%), LLA (n=17, 25%), IA (n=13, 19.12%) and ULA (n=6, 8.82%). We also made a study of the number of vascular territories affected: 1 vascular territory (n=13, 19.12%), 2 territories (n=22, 32.35%), 3 territories (n=18, 26.47%), 4 territories (n=12, 17.65%) and more than 4 territories (n=3, 4.41%). Likewise, a comparative study between both sexes was conducted, in which only statistical significance was achieved in the involvement of ULA, which was more frequent in men (table 1). Conclusions In patients with GCA the involvement of TA is very frequent, followed by the SAT and the AA. To a lesser extent, the AA and the LLA vessels are affected. The involvement of the IA and the ULA vessels is less frequent; the latter more frequently in men. On the other hand, the involvement of 2–3 vascular territories are the most frequent patterns. References [1] Loricera J, Blanco R, Hernandez JL, et al. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol. 2015;34:372–377. [2] Loricera J, Blanco R, Hernandez JL, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015;33:S19–31. [3] Loricera J, Blanco R, Hernandez JL, et al. Tocilizumab in giant cella arteritis: Multicenter open-label study of 22 patients. Semin Arthritis Rheum. 2015;44:717–723. Disclosure of Interest None declared

Background Giant cell arteritis (GCA) is a large vessel vasculitis that has a special predilection for extracranial branches of the external carotid artery. Among its most fearsome complications is visual affectation. Tocilizumab (TCZ) is a monoclonal antibody directed against the interleukin 6 receptor that has shown utility in the treatment of GCA. Objectives Our aim was to assess the evolution of visual clinic in patients with GCA treated TCZ. Methods Retrospective multicentre study of 20 GCA patients with visual involvement treated with TCZ. The efficacy of this drug on visual symptoms was evaluated. Results We evaluated 20 patients (14 women and 6 men) with a mean age ±SD of 73.7±10.1 years with GCA and visual symptoms. In total there were 23 affected eyes. The symptoms reported were: unilateral blindness (n=6), unilateral blurred vision (n=6), unilateral amaurosis fugax (n=3), unilateral hemianopsia (n=2), bilateral blindness (n=1), bilateral blurred vision (n=1), bilateral hemianopsia (n=1). Before starting treatment with TCZ all patients had received high doses of prednisone, with a mean ±SD of 54.2±13.8 mg/day (range of doses: 40–80 mg/day). In addition, 9 of them also received intravenous corticosteroid boluses. In addition, 13 patients received traditional immunosuppressants: methotrexate (MTX) (n=12), cyclophosphamide (n=2), leflunomide (n=1) and azathioprine (n=1). Regardless of corticosteroids, TCZ was administered as monotherapy in 14 patients, while in 6 it was administered in combination with MTX. The TABLE shows the evolution of the visual affectation of these patients. Throughout a median follow-up [RIC] of 94–18 months, none of the 7 patients who had a blindness regained vision. The 2 patients who presented unilateral hemianopsia recovered vision. The patient with bilateral hemianopsia and the patient with bilateral blurred vision experienced a partial improvement. The rest of the patients achieved a complete recovery. Conclusions Although TCZ seems to be also useful in the treatment of visual manifestations of ACG, once blindness is established, it does not seem to be effective. Disclosure of Interest None declared