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BackgroundGraves’ orbitopathy (GO) is an autoimmune disease of the retroocular tissues that occurs in patients with Graves’ disease. Tocilizumab (TCZ), an interleukin (IL)-6 inhibitor has been used in patients with glucocorticoid (GC)-resistant Graves’ orbitopathy with good results. We have previously reported results obtained in 15 OG patients treated with TCZ where a decrease in mean CAS from 4.9 to 1.7 was observed. Sarilumab, another IL-6 inhibitor, is indicated for the treatment of rheumatoid arthritis and has been used in other pathologies as an off-label use, similar to TCZ, on the grounds that they share a common mechanism of action.ObjectivesTo analyse the effectiveness of Sarilumab treatment in patients with GC-resistant OG in routine clinical practice.MethodsA descriptive retrospective observational study of a series of patients with moderate-severe active OG on Sarilumab treatment was performed. Medical records of patients who had received at least 1 cycle of Sarilumab treatment were reviewed. Demographic and disease data were collected. Effectiveness was assessed by CAS (Clinical Activity Score) reduction before and at 3-6 months of treatment. A reduction of CAS≥ 2 points together with obtaining inactivity (CAS < 3) was considered a favourable response. The study was approved by Research Ethics Committee.ResultsIn the rheumatology department of the Hospital Virgen Macarena, 25 patients were treated with IL-6 inhibitors, 20 with tocilizumab and 5 with Sarilumab. Five patients were included, 2 women and 3 men, with a mean age of 43 years. All but one had bilateral ocular involvement. 2 patients were active smokers, 1 ex-smoker and 2 non-smokers. Two patients were diabetic, none of them had retinopathy. The mean time of disease until their evaluation in Rheumatology was 3.5 years. All had been treated with antithyroid drugs, 1 with Radioiodine and one patient had undergone thyroidectomy. 100% of patients had an active moderate-severe orbitopathy, all had been treated with local measures, 3 with selenium and none had undergone rehabilitative surgery. Three patients had been on at least one course of intravenous steroids. Patients received Sarilumab at a dose of 200 mg subcutaneous injection every 15 days for a mean of 11 months (range 7-21 months), 100% in monotherapy. The mean initial CAS was 5/7 and the final CAS was 2.75/10. The CAS score was reduced by 3 points on average. 100% had a favourable response and achieved inactivity, also reducing the severity of the disease. Furthermore, there was an improvement in thyroid function parameters and a reduction in antibody titters against TSH receptor and anti-thyroid peroxidase. All patients were continuing treatment except for one patient who had discontinued treatment due to clinical remission. No adverse events were recorded except for mild neutropenia in one patient, which did not require dose reduction. No patient required subsequent immunosuppressive treatment or surgery.ConclusionSarilumab treatment in patients with moderate-severe active OG appears to be an effective and safe therapeutic alternative, clinically similar to that obtained with TCZ in our series. This is the first published series of Sarilumab use in OG patients.References[1]Bartalena L, Kahaly GJ, Baldeschi L, et al. THE 2021 EUROPEAN GROUP ON GRAVES’ ORBITOPATHY (EUGOGO) CLINICAL PRACTICE GUIDELINES FOR THE MEDICAL MANAGEMENT OF GRAVES’ ORBITOPATHY. Eur J Endocrinol. 2021; EJE-21-0479.R1. doi: 10.1530/EJE-21-0479.[2]Sánchez-Bilbao L, Martínez-López D, Revenga M, et al. Anti-IL-6 Receptor Tocilizumab in Refractory Graves’ Orbitopathy: National Multicenter Observational Study of 48 Patients. J Clin Med. 2020;9(9):2816.[3]L. Fernández-Fuente-Bursón, F.J.Toyos-Sáenz-Miera, M.D. Ruiz-Montesino, et al. EFECTIVIDAD DEL TRATAMIENTO CON ANTI-INTERLEUCINA 6 SUBCUTÁNEA EN PACIENTES CON ORBITOPATÍA DE GRAVES MODERADA-GRAVE ACTIVA REFRACTARIA A TERAPIA CONVENCIONAL. Poster presented at the congress: 36º Congreso Nacional Sociedad Española de Reumatología; 2020 Oct 20-24; virtual.Disclosure of InterestsNone declared

Background:Factors associated with the development of chronic heart failure (CHF) in systemic lupus erythematosus (SLE) have received little attention. On the other hand, recent data from the use of hydroxychloroquine in the treatment of SARS-CoV-2 infection during the COVID19 pandemic have cast some doubts on its cardiological safety.Objectives:To identify factors associated to CHF in SLE.Methods:Retrospective cross-sectional study, including all patients with SLE (≥4 ACR-1997 criteria) recruited in RELESSER registry. The objectives and methodology of the registry have been described previously (1). CHF was defined according to the Charlson index item. Patients with CHF before diagnosis of SLE were excluded. Cumulative damage was measured with the SLICC/ACR index, excluding cardiovascular (CV) items (mSDI). Multivariate analysis exploring factors associated with CHF was carried out.Results:117 patients (3% of the entire cohort) with SLE and CHF and 3,506 controls with SLE without CHF were included. 90% were women. Disease duration: mean (SD), 120.2 (87.7) months. CHF appeared after a median (P25-P75) of 9.40 (4.2-18.3) years from SLE diagnosis. Patients with CHF were older (59.8 ± 18.2 vs. 46.2 ± 4.3). In the bivariate analysis, the association of CHF with greater severity [Katz severity index: median (IQR): 4 (3-5) vs. 2 (1-3)], damage [mSDI: 3 (2-4) vs 0 (0-1)], comorbidity [modified Charlson- excluding CV items: 4 (3-6) vs 1(1-3)] and both CV (37.5% vs 6.7%) and overall mortality (43.2% vs 4.7%) (pTable 1.Congestive heart failure associated factors (multivariable analysis)Odds Ratio95% CIP-valueSex (female)0.460.25 - 0.880.0147Ischaemic cardiopathy7.964.01 - 15.48Cardiac arrhythmia7.384.00 - 13.42Pulmonary hypertension3.711.84 - 7.250.0002Cardiac valvulopathy6.333.41 - 11.62Hospitalization (due to SLE)3.741.81 - 8.650.0008Calcium or vitamin D5.292.07 - 16.860.0015Antimalarials0.280.17 - 0.45mSDI *1.291.16 - 1.44*mSDI = modified SLICC/ACR damage index (without cardiovascular items)Conclusion:- CHF is a rather late complication of SLE.- Patients with SLE and CHF have more severe SLE, with greater refractoriness to SLE treatments and higher overall mortality.- Treatment with antimalarials, as routinely used in SLE patients, is not only safe to heart, but even appears to have a cardioprotective effect.References:[1]Rúa-Figueroa I, López-Longo FJ, Calvo-Alén J, et al. National registry of patients with systemic lupus erythematosus of the Spanish Society of Rheumatology: objectives and methodology. Reumatol Clin. 2014;10(1):17-24.Acknowledgements:Research Unit of Spanish Society of RheumatologyDisclosure of Interests:None declared

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:The antisynthetase syndrome (SAS) is characterized by the presence of antisynthetase antibodies, anti-JO1, PL7 y PL12 are the most common; and the classic triad of myositis, arthritis, and diffuse interstitial lung disease (ILD)1. Most patients present incomplete forms and the severity of the ILD determines the prognosis of the disease2.Objectives:to analyze epidemiological, clinical and serological characteristics and treatments used in a cohort of patients with SAS.Methods:descriptive study of review of medical records. Data were collected from 15 patients with SAS followed in the Rheumatology and Pneumology consultations of the Virgen Macarena Hospital (Seville) in the last 10 years. The analysis was carried out using the R software.Results:15 patients were included, 8 men and 7 women. The median age was 56 years (33-77). Seven patients (47%) used to smoke. Four patients (27%) met the classical triad. All of them presented ILD and 8 patients (53%) had arthritis and / or myositis. Five (33%) had mechanic’s hands and six of them (40%) presented Raynaud. Seven (47%) suffered from dyspnea before the SAS diagnosis. The median diagnostic delay was 1 month (0-43). Seven (47%) patients had anti-JO1, 1 (7%) anti-PL7, 2 (13%) anti-PL12 and 2 (13%) patients anti-Ro52. Radiological patterns detected by HRCT were: 5 (33%) NINE, 4 (37%) NIU and 6 (40%) others. The initial treatment included mostly (66%) glucocorticoids (GC) and one or more cFAME. In maintenance, mycophenolate was used in 7 patients (47%), cyclosporine 5 (33%), cyclophosphamide in 3 cases (20%), azathioprine in 3 patients (20%) and methotrexate in 3 of them (20%). Four (37%) patients required a combination of DMARDs and 2 cases needed (13%) biological therapy, Rituximab and Tocilizumab. Changes in the mean value of the initial respiratory function tests (FVC1 and DLCO1) and during follow-up (FVC2 and DLCO2) were not relevant (FVC1 81.5% [42-110], FVC2 81% [59-115]; DLCO1 83% [10-112], DLCO2 80.5% [47-108]). Nine patients (60%) remained clinically stable and 3 patients (20%) progressed radiologically. Four patients died from ILD progression.Conclusion:In this study, the incomplete diagnosis of SAS predominated. The most detected antibody was anti-JO1. ILD is present in all cases, with NINE being the most frequent pattern so multidisciplinary management is necessary. Most used treatments were GC and FAMES combined, some cases required biological therapy.References:[1]Irazoque F, et al. Epidemiology, etiology and classification. Reumatol Clin. 2009;5:2-5.[2]Johnson C, et al. Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. Respir Med. 2014; 108(10):1542-8.Disclosure of Interests:None declared

Background:Graves’ Orbitopathy (OG) is the main extrathyroid manifestation of Graves’ disease (GD). Up to 25% of patients have moderate-severe inflammatory activity with a risk of major sequelae. Intravenous (i.v.) glucocorticoids (GC) pulses are the standard therapy. In refractory cases, several classic and some biological immunosuppressantsare used, obtaining variable responses. Interleukin 6 (IL6) is involved in the pathogenesis of OG, which has justified the off-label treatment with Tocilizumab (TCZ)1, whose use by route i.v. has shown favorable results in published small case series and a single multicenter trial2.Objectives:To analyse the ocular effectiveness of anti-IL6 therapy used subcutaneously (s.c.) in patients with moderate-severe active refractory OG in usual clinical practice.Methods:Retrospective descriptive observational study of a series of cases of moderate-severe OG patients treated with anti-IL6 s.c. The patient medical records of those who had received at least 1 cycle of anti-IL6 treatment were reviewed (December 2013-December 2019). The primary effectiveness outcome was the change of the Clinical Activity Score (CAS). Favorable response was considered: reduction of CAS≥2 points together with obtaining inactivity (CAS Results:12 of the 15 patients (80%) were women with a mean of 50.27 years (21-72). 60% (n=9) had smoking history, 40% (n=6) active. 26.7% (n=4) were diabetic, all without retinopathy. 100% of patients received imidazole antithyroid treatment. 46.7% (n=7) required β-blockers and 20% (n=3) diuretics. 66.7% thyroidectomy (n=10) and 20% (n=3) decompressive eye surgery and/or blepharoplasty were performed. Thyroid and ocular radiotherapy were used in 2 patients. 3 patients received botox. 80% (n=12) of them had previously received GC. 93.3% (n=14) were naïve to biological therapy, only 1 patient previously used Rituximab. All except one patient who was treated with SRL received TCZ as IL6 therapy. A significant favorable response was obtained in 100% of the patients (p=0.008), decreasing CAS average from 4.9 (2-7) to 1.7 (0-2) at the end of the therapy [Figure 1]. The severity of the OG changed from being moderate in 72.7% of the patients to mild in 66.7% of the total. The median time to inactivity was 8 months (2-15). 73.3% (n=11) of the patients finished the treatment reaching inactive OG, the rest (although inactive) maintained therapy. After 6 months, 100% of those who completed the treatment remained inactive with average CAS of 1.3 (0-2). Smoking did not influence the response, nor any other variable collected. Adverse events appeared in 26.7% (n=4) of the cases, all of them mild and without widrawal.Conclusion:Treatment with anti-IL6 s.c. steadily decreases the clinical activity measured by CAS in patients with moderate-severe refractory OG, despite poor prognosis factors (such as smoking), with a good safety profile.References:[1]Taylor PN, Zhang L, Lee RWJ, Muller I, Ezra DG, Dayan CM, et al. New insights into the pathogenesis and nonsurgical management of Graves’ orbitopathy. Nat Rev Endocrinol. 2020 Feb;16(2):104-116.[2]Perez-Moreiras JV, Gomez-Reino JJ, Maneiro JR, Perez-Pampin E, Romo Lopez A, Rodríguez Álvarez FM, et al. Efficacy of Tocilizumab in Patients With Moderate-to-Severe Corticosteroid-Resistant Gravesˊ Orbitopathy: A Randomized Clinical Trial. Am J Ophthalmol. 2018;195:181–90.Disclosure of Interests:None declared

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 Cryopyrin-associated periodic syndrome (CAPS) comprises a group of rare, but severe, autoinflammatory diseases, characterized by urticaria, periodic fever, central nervous system inflammation, arthropathy, and increased risk of amyloidosis. In a recent trial the use of subcutaneous doses of 150 mg of canakinumab every 8 weeks was associated with complete control of clinical manifestations and laboratory parameters in patients with CAPS. Objectives The aim of this study was to verify efficacy and safety of the drug in clinical practice. Methods Retrospective longitudinal observational study, which included all patients diagnosed with CAPS in a tertiary hospital. Demographic and disease characteristics of all patients were collected. The decision to treat was performed by their rheumatologists. Clinical and laboratory variables at last follow-up were compared with those registered at Canakinumab treatment baseline. Percentages were obtained for qualitative variables and means with standard deviation (SD) for quantitative variables. Comparison between disease activity before and after therapy was performed with a t -student test. Results 10 genetically proved CAPS patients were included. 9 of them presented with the typical MWS phenotype and one of them patients with an overlapping MWS/FCAS phenotype. Seven of the patients belonged to the same family. All 10 patients were heterozygous carriers of different mutations in the NLRP3 gene: p.Thr-348-Met in exon 3, D303N in all family affected members, a deletion of bp (A) in exon 3, a mutation not described before, and finally, pR260W. Because disease activity, 50% of the patients were treated with a subcutaneous dose of 150 mg every 8 weeks of Canakinumab. Clinical and laboratory parameters of those patients are shown in Table 1. Both clinical and laboratory parameters responded quickly to that therapy. The mean decrease in CRP levels was 83.2 mg/L (SD 64.1) (p 0.04) in the levels of ESR of 40.8 mm/h (SD 24.9) (p 0,02). The mean increase in hemoglobin levels was 2.5 points (SD 2.62) (p 0.1) and the mean decrease in the platelet count was 161250 (SD 158838) (p 0.135). One of the patients is in a reduced dose schedule, receiving canakinumab every 10 weeks. No patients presented with any adverse events during follow up. Conclusions The use of canakinumab in daily practice is associated with persistent satisfactory control of disease activity. Here 10 patients with different mutations are presented, including a not previously reported mutation. References Muckle TJ, et al. QJM 1962;31:235-48. Calvo I, et al. Med Clin (Barc) 2011;136(Supl 1):16-21. Houten SM, et al. Cell Mol Life Sci 2003;60:1118-34. Lachmann HJ, et al. N Engl J Med2009 Jun;360(23):2416-25 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5712