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Whipple’s disease (WD) is a rare infectious disease with a wide clinical spectrum. Associated thrombotic manifestations are not well described in WD, only related to ‘stroke-like syndrome’. We present a case of a 39-year-old man with a 1-year history of self-limited episodes of fever, associated with generalised adenopathies and recurrent superficial and deep venous thrombosis events, which have resorted four times despite the anticoagulant treatment. Finally, the patient is diagnosed with WD. Following treatment the patient improved in his general condition, and no more episodes of fever neither thrombosis appeared during a follow-up of more than 3 years.

Two clinical subsets of giant cell arteritis have been identified with different histological and CT findings. However, PET/CT findings have not been compared with temporal artery biopsy (TAB).The aims of this study were to describe clinical and histological findings in patients with giant cell arteritis according to the presence or absence of aortitis in PET/CT at the disease diagnosis, and to identify independent factors related to aortic involvement.Patients were included and followed prospectively. Clinical symptoms and TAB findings were recorded. PET/CT was performed in the first 10 days of steroid therapy. Aortitis was defined if a grade 3 uptake on visual analysis was present on arterial wall. Clinical and histological variables were compared according to the presence or absence of aortitis on PET/CT. Multivariate analysis was performed to identify independent factors related to the presence of aortitis.Twenty-seven patients (median age, 77.6 years) were included. PET/CT was performed with a median delay of 5.0 days. Aortitis was observed in 8 patients. Patients with aortitis were younger (69.9 vs 83.7 years, P = 0.04) and had less frequently ischemic manifestations (25.0% vs 84.2%, P = 0.006) than patients without aortitis. Giant multinucleated cells were more frequent on TAB from patients with aortitis (71.4% vs 16.7%), and its presence was an independent risk factor for the occurrence of aortic involvement on PET/CT (odds ratio, 12.2; P = 0.046).Our study shows that giant cells on TAB are associated with the presence of aortitis on PET/CT. Patients with aortic involvement are younger and show less frequently ischemic manifestations.

Sjogren's syndrome is an autoimmune disease that involves exocrine glands. The most characteristic symptoms consist of the sicca syndrome (including xerostomia and dry eye - xerophtalmia), but can involve multiple organs. The extraglandular involvement determines the prognosis. It is typically associated with the presence of antinuclear antibodies, including Ro-60 antibodies. Pulmonary involvement appears as bronchiectasis and/or interstitial pneumonia. Considering its high prevalence, it must be ruled out in all patients with respiratory symptoms by performing pulmonary function tests and high-resolution computed tomography of the chest. Evaluation can be completed with a transbronchial biopsy if diagnostic doubts persist. Treatment includes steroid therapy, inmunosupressive or antifibrotic drugs, or biological therapy. In selected cases pulmonary transplantation must be considered.

Objective To assess the potential diagnostic utility of advanced lymphocyte profiling to differentiate between primary Sjögren’s Syndrome (pSS) and non-Sjögren Sicca syndrome. Methods Distribution of peripheral lymphocyte subpopulations was analysed by flow cytometry in 68 patients with pSS, 26 patients with sicca syndrome and 23 healthy controls. The ability to discriminate between pSS and sicca syndrome was analysed using the area under the curve (AUC) of the receiver operating characteristic curve of the different lymphocyte subsets. Results The ratio between naïve/memory B cell proportions showed an AUC of 0.742 to differentiate pSS and sicca syndrome, with a sensitivity of 76.6% and a specificity of 72% for a cut-off value of 3.4. The ratio of non-switched memory B cells to activated CD4+ T cells percentage (BNSM/CD4ACT) presented the highest AUC (0.840) with a sensitivity of 83.3% and specificity of 81.7% for a cut-off value Conclusion Patients with pSS show a profound imbalance in the distribution of circulating T and B lymphocyte subsets. The ratio BNSM/CD4ACT is useful to discriminate between pSS and sicca syndrome.

Summary Long-term observation of patients with ANCA-associated vasculitis (AAV) allows the identification of different longitudinal patterns of ANCA levels during follow-up. This study aimed to characterize these patterns and to determine their prognostic significance. All ANCA determinations performed in two university hospitals during a 2-year period were retrospectively reviewed. Patients were included in the analysis if they had high titers of anti-myeloperoxidase (anti-MPO) or anti-proteinase 3 (anti-PR3) antibodies at least once, ≥ 5 serial ANCA determinations and AAV diagnosed by biopsy or American College of Rheumatology (ACR) classification criteria. Patients’ time–course ANCA patterns were classified as monophasic, remitting, recurrent or persistent. Associations between ANCA patterns and prognostic variables (relapse rate and renal outcome) were analysed by univariate and multivariate statistics. A total of 99 patients [55 with microscopic polyangiitis (MPA), 36 with granulomatosis with polyangiitis (GPA) and eight with eosinophilic granulomatosis with polyangiitis (EGPA)] were included. Median follow-up was 9 years. Among patients diagnosed with MPA or GPA, recurrent or persistent ANCA patterns were associated with a higher risk of clinical relapse [hazard ratio (HR) = 3·7, 95% confidence interval (CI) = 1·5–9·1 and HR = 2·9, 95% CI = 1·1–8·0, respectively], independently of clinical diagnosis or ANCA specificity. In patients with anti-MPO antibodies, the recurrent ANCA pattern was associated with worsening renal function [odds ratio (OR) = 5·7, 95% CI = 1·2–26·0]. Recurrent or persistent ANCA patterns are associated with a higher risk of clinical relapse. A recurrent ANCA pattern was associated with worsening renal function in anti-MPO-associated vasculitis.

Objective To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P 65 years, P Conclusion The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Objective: To compare the accuracy of the Birmingham Vasculitis Activity Score (BVAS), version 3, and the Five Factor Score (FFS), version 1996 and version 2009, to assess survival in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Methods: A total of 550 patients with AAV (41.1% with granulomatosis with polyangiitis, 37.3% with microscopic polyangiitis, and 21.6% with eosinophilic granulomatosis with polyangiitis), diagnosed between 1990 and 2016, were analyzed. Receiver operating characteristic (ROC) curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores. Results: Overall mortality was 33.1%. The mean ± SD BVAS at diagnosis was 17.96 ± 7.82 and was significantly higher in nonsurvivors than in survivors (mean ± SD 20.0 ± 8.14 versus 16.95 ± 7.47, respectively; P < 0.001). The mean ± SD 1996 FFS and 2009 FFS were 0.81 ± 0.94 and 1.47 ± 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean ± SD 1996 FFS 1.17 ± 1.07 versus 0.63 ± 0.81 [P < 0.001] and 2009 FFS 2.13 ± 1.09 versus 1.15 ± 1.05 [P < 0.001], respectively). Mortality rates increased according to the different 1996 FFS and 2009 FFS categories. In multivariate analysis, BVAS, 1996 FFS, and 2009 FFS were significantly related to death (P = 0.007, P = 0.020, P < 0.001, respectively), but the stronger predictor was the 2009 FFS (hazard ratio 2.9 [95% confidence interval 2.4–3.6]). When the accuracy of BVAS, 1996 FFS, and 2009 FFS to predict survival was compared in the global cohort, ROC analysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009 FFS had the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001 and when assessing the 1-year, 5-year, and long-term mortality. Correlation among BVAS and 1996 FFS was modest (r = 0.49; P < 0.001) but higher than between BVAS and the 2009 FFS (r = 0.28; P < 0.001). Conclusion: BVAS and FFS are useful to predict survival in AAV, but the 2009 FFS has the best prognostic accuracy at any point of the disease course.

Sjögren's syndrome is an autoimmune disease that involves exocrine glands. The most characteristic symptoms consist of the sicca syndrome (including xerostomia and dry eye - xerophtalmia), but can involve multiple organs. The extraglandular involvement determines the prognosis. It is typically associated with the presence of antinuclear antibodies, including Ro-60 antibodies. Pulmonary involvement appears as bronchiectasis and/or interstitial pneumonia. Considering its high prevalence, it must be ruled out in all patients with respiratory symptoms by performing pulmonary function tests and high-resolution computed tomography of the chest. Evaluation can be completed with a transbronchial biopsy if diagnostic doubts persist. Treatment includes steroid therapy, inmunosupressive or antifibrotic drugs, or biological therapy. In selected cases pulmonary transplantation must be considered.

To analyse the risk of ischaemic events in patients with newly diagnosed giant cell arteritis (GCA) according to PET/CT findings. PET/CT was performed during the first 10 days of steroid therapy. Clinical manifestations at diagnosis, and physical examination and PET/CT findings were recorded and compared according to the presence or absence of ischaemic symptoms at disease onset. Analysed territories included the ascending aorta, aortic arch, descending aorta, abdominal aorta, carotid arteries, brachiocephalic trunk, vertebral arteries, subclavian arteries and axillary arteries. The study group comprised 30 patients with a median age of 80.8 years. Of these patients, 21 (70%) reported ischaemic symptoms at diagnosis, and 13 (43.3%) had permanent visual loss. Of the 30 patients, 77.8% showed large vessel vasculitis (including aortic and vertebral artery involvement) on PET/CT, and 60% had isolated involvement of the vertebral territory. Vertebral arteries were more frequently involved in patients with ischaemic symptoms (OR 5.0, 95% CI 0.99–24.86, p = 0.051). The presence of vertebral artery involvement in the absence of aortic involvement was associated with the presence of ischaemic manifestations (Fisher’s exact test, p = 0.001). The presence of aortitis was found to protect against the development of permanent visual loss (OR 19.0, 95% CI 2.79–127.97, p = 0.001). Our findings suggest an association between the vascular pattern on PET/CT at the time of GCA diagnosis and the risk of ischaemic events.

Arteritis de Takayasu; Tocilizumab; Teràpia biològica Arteritis de Takayasu; Tocilizumab; Terapia biológica Takayasu’s arteritis; Tocilizumab; Biological therapy Objective: To assess the efficacy and safety of tocilizumab (TCZ) in Caucasian patients with refractory Takayasu’s arteritis (TAK) in clinical practice. Methods: A multicenter study of Caucasian patients with refractory TAK who received TCZ. The outcome variables were remission, glucocorticoid-sparing effect, improvement in imaging techniques, and adverse events. A comparative study between patients who received TCZ as monotherapy (TCZMONO) and combined with conventional disease modifying anti-rheumatic drugs (cDMARDs) (TCZCOMBO) was performed. Results: The study comprised 54 patients (46 women/8 men) with a median [interquartile range (IQR)] age of 42.0 (32.5–50.5) years. TCZ was started after a median (IQR) of 12.0 (3.0–31.5) months since TAK diagnosis. Remission was achieved in 12/54 (22.2%), 19/49 (38.8%), 23/44 (52.3%), and 27/36 (75%) patients at 1, 3, 6, and 12 months, respectively. The prednisone dose was reduced from 30.0 mg/day (12.5–50.0) to 5.0 (0.0–5.6) mg/day at 12 months. An improvement in imaging findings was reported in 28 (73.7%) patients after a median (IQR) of 9.0 (6.0–14.0) months. Twenty-three (42.6%) patients were on TCZMONO and 31 (57.4%) on TCZCOMBO: MTX (n = 28), cyclosporine A (n = 2), azathioprine (n = 1). Patients on TCZCOMBO were younger [38.0 (27.0–46.0) versus 45.0 (38.0–57.0)] years; difference (diff) [95% confidence interval (CI) = -7.0 (-17.9, -0.56] with a trend to longer TAK duration [21.0 (6.0–38.0) versus 6.0 (1.0–23.0)] months; diff 95% CI = 15 (-8.9, 35.5), and higher c-reactive protein [2.4 (0.7–5.6) versus 1.3 (0.3–3.3)] mg/dl; diff 95% CI = 1.1 (-0.26, 2.99). Despite these differences, similar outcomes were observed in both groups (log rank p = 0.862). Relevant adverse events were reported in six (11.1%) patients, but only three developed severe events that required TCZ withdrawal. Conclusion: TCZ in monotherapy, or combined with cDMARDs, is effective and safe in patients with refractory TAK of Caucasian origin. The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was partially supported by RETICS Programs, RD08/0075 (RIER), RD12/0009/0013 and RD16/0012 from “Instituto de Salud Carlos III” (ISCIII) (Spain). However, this research did not receive any specific grant from funding agencies in the commercial or not-for-profit sectors.

Objective To define the pattern of cardiac involvement in eosinophilic granulomatosis and polyangiitis (EGPA) and propose an algorithm for heart disease screening. Methods This was a retrospective study of EGPA patients attending a specialized vasculitis clinic (1989–2016). Clinical characteristics and cardiovascular evaluation (CE) results (serum troponin, ECG, echocardiography and cardiac magnetic resonance) were collected and compared according to symptoms and inflammatory cardiac disease (ICD). Results A total of 131 EGPA patients were included, of whom 96 (73%) had undergone CE. The median (interquartile range) age was 50 (38–58) years and 36% showed ANCA+. Asthma preceded diagnosis by a median of 97 (36–240) months. Among the 96 patients who underwent CE, 43% were symptomatic, with dyspnea (47%) and chest pain (29%) being the predominant symptoms. In asymptomatic patients, CE reported abnormalities in 45% of cases, with a subsequent earlier diagnosis (4 vs 11 months). Overall, 27 patients had EGPA-related ICD (EGPA-rICD) that was already present at diagnosis in 20 cases, preceded it in 2 cases and developed later in 5 cases. EGPA-rICD patients were younger (46 vs 50 years; P = 0.04), had more frequently abnormal ECG (30.8 vs 2.1%; P Conclusion In our study, 45% of asymptomatic patients had an abnormal baseline cardiac evaluation, which allowed an earlier diagnosis of cardiac disease. We recommend prompt cardiac screening in all EGPA patients, instead of a symptoms-guided algorithm.

Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18F-florbetapir. We hypothesize that combining whole body PET/CT with 18F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls. Target-to-background ratio was computed for multiple organ using maximum standardized uptake values. Organ involvement was described (standardized techniques versus PET) according to different kinds of amyloidosis showing promising results in AA and AL types. Heart involvement showed poorer results when compared to tongue, lung or thyroid gland. Multiple organ involvement in patients previously labelled as having negative organ affectation could be identified. This is the first study to evaluate the utility of 18F-florbetapir in the assessment of the global extension of disease. Our results show that this technique is useful for its diagnosis.

Background:Tocilizumab (TCZ) has shown to be effective for large vessel vasculitis including Takayasu arteritis (TAK) (1-3). Most evidence in TAK comes from Asian patients. However, white patients seem to have different clinical and prognostic features.Objectives:Our aims were to: a) assess the efficacy and safety of TCZ in white patients with refractory TAK, b) determine if clinical improvement correlates with imaging outcomes, c) compare TCZ in monotherapy (TCZMONO) vs combined with conventional immunosuppressive drugs (TCZCOMBO)Methods:Multicenter study of white patients with refractory TAK who received TCZ.Outcomes variables were remission, glucocorticoid-sparing effect, improvement in imaging techniques, and adverse events. A comparative study between patients who received TCZMONO and TCZCOMBO was performed.Results:54 patients (46 women/8 men; median age 42.0 [32.5-50.5] years). TCZ was started after 12.0 [3.0-31.5] months since TAK diagnosis. Remission was achieved in 12/54 (22.2%), 19/49 (38.8%), 23/44 (52.3%) and 27/36 (75%) at 1, 3, 6 and 12 months, respectively. Prednisone dose was reduced from 30.0 [12.5-50.0] to 5.0 [0.0-5.6] mg/day at 12 months (Table 1). 10 (26.3%) of the 38 patients in whom an imaging follow-up test was performed showed no radiographic improvement after a median of 9.0 [6.0-14.0] months. 4 of them were in clinical remission.23 (42.6%) patients were on TCZMONO and 31 (57.4%) on TCZCOMBO: MTX (n=28), cyclosporine A (n=2), azathioprine (n=1). Patients on TCZCOMBO were younger (38.0 [27.0-46.0] vs 45 [38.0-57.0] years; p= 0.048), with a trend to longer TAK duration (21.0 [6.0-38.0] vs 6.0 [1.0-23.0] months; p= 0.08) and higher C-reactive protein (2.4 [0.7-5.6] vs 1.3 [0.3-3.3] mg/dL; p=0.16). Despite these differences, similar outcomes were observed in both groups (log rank p=0.862) (Figure 1). Relevant adverse events were reported in 6 (11.1%) patients, but only 3 developed severe events that required TCZ withdrawal.Table 1.Baselinen=54Month 1N=54Month 3N=49Month 6N=44Month 12N=36Clinical remission, n (%)12 (22.2)19 (38.8)23 (52.3)27 (75.0)Laboratory improvementCRP (mg/dL), median [IQR]1.5 [0.5-3.5]0.2 [0.1-0.7]*0.2 [0.5-0.5]*0.2 [0.1-0.5]*0.1 [0.0-0.4]*ESR (mm/1sthour), median [IQR]30.5 [8.7-52.7]7.0 [3.0-14.0]*4.5 [2.0-8.0]*5.0[2.0-6.0]*4.0 [2.0-9.5]*Hemoglobin (g/dL), mean ± SD12.4 ±1.513.0 ±1.2*13.0 ±1.4*13.2 ±1.5*12.9 ±1.6*Prednisone dose, median [IQR]30.0 [12.5-50.0]20.0 [10.0-30.0]*10.0 [5.0-20.0]*5.0 [5.0-10.5]*5.0 [0.0-5.6]*CRP: C-Reactive Protein; ESR: Erythrocyte Sedimentation Rate; IQR: interquartile range; n: number. *pConclusion:TCZ is effective and safe in white patients with refractory TAK. A discordance between clinical and imaging activity assessment may exist.References:[1]Prieto Peña D et al. Clin Exp Rheumatol 2020 Nov 27. PMID: 33253103.[2]Loricera J, et al. Clin Exp Rheumatol 2016; 34:S44-53. PMID: 27050507.[3]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019; 49:126-35. PMID: 30655091Disclosure of Interests:Diana Prieto-Peña Grant/research support from: DP-P has received research support from UCB Pharma, Roche, Sanofi, Pfizer, AbbVie and Lilly., 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: None declared, Esteban Rubio Romero: None declared, SALVADOR GARCÍA MORILLO: None declared, Mauricio Minguez: None declared, Cristina Fernández-Carballido: None declared, Eugenio de Miguel: None declared, Sheila Melchor: None declared, Eva Salgado-Pérez: None declared, Beatriz Bravo: None declared, Susana Romero-Yuste: None declared, Juan 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, JOSE LUIS ALONSO VALDIVIESO: None declared, Pérez Sánchez Laura: None declared, Roldán Molina Rosa: None declared, Nagore Fernández-Llanio: None declared, Ricardo Gómez de la Torre: None declared, Silvia Suarez: None declared, María Jesús Montesa: None declared, Monica Delgado Sanchez: None declared, J. Loricera: None declared, Belén Atienza-Mateo: None declared, Santos Castañeda: None declared, Miguel A González-Gay Grant/research support from: MAG-G received grants/research supports from Abbvie, MSD, Jansen and Roche and had consultation fees/participation in company sponsored speaker´s bureau from Abbvie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Ricardo Blanco Grant/research support from: RB received grants/research supports from Abbvie, MSD and Roche, and had consultation fees/participation in company sponsored speaker´s bureau from Abbvie, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD.

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 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: Behcet disease (BD) is considered a systemic vasculitis according to the Chapel-Hill classification, which occurs most frequently between latitudes 30 and 45° north and in the area of the Old Silk Route. Turkey has the highest prevalence, followed by Japan. In Spain, a prevalence of 7.5 cases per 100000 persons has been estimated. The epidemiological and clinical characteristics of European patients with BD vary with respect to those of the Turkish and Japanese cohorts. Objectives: Description of clinical features and treatment received in a cohort of patients diagnosed with BD in an internal Medicine Unit of a tertiary centre from Barcelona. Methods: Retrospective, observational study. Epidemiological, clinical and laboratory data were obtained from clinical charts. SSPS package was used to perform statistical analysis. Results: 132 patients (56.6% men) diagnosed over the last 30 years and followed-up until the censoring data were included. 112 (84.8%) were Caucasians, 15 (11.4%) from North africa, 4 (3%) asiatic and 1 (0.8%) from South america. 2 (1.5%) patients had a family history of BD and 9 patients (6.8%) a family history of other rheumatic diseases. 43.9% (58 patients) were HLA-B51. Oral or genital ulcers were present in 131 (99.2%) patients and skin involvement in 106 (80%) cases, 44.6% had erythema nodosum, 59% had acne-like lesions and 9% had cutaneous vasculitis. Ocular involvement was observed in 69 cases (52.3%): 27 patients unilateral and 8 bilateral anterior uveitis; 14 unilateral and 3 bilateral posterior uveitis; 10 patients unilateral and 14 bilateral panuveitis; 19 unilateral retinal vasculitis and 12 bilateral. Neurological involvement was present in 37 (28%) patients: 10 parenchimal disease and 17 cases non-parenchymal disease (aseptic meningitis or vasculitis); 8 patients benign intracranial hypertension, and 3 had dural sinus thrombosis. Articular involvement was recorded in 79 (59.8%) patients (it was observed 25 monoarthritis, 24 oligoarthritis, 6 poliarthritis and 58 patients had arthralgia). Vascular involvement was present in 43 (32.6%) cases: deep venous thrombosis in 38 patients (11 cases in locations other than the extremities); pulmonary embolism in 7; 21 thrombophlebitis; and 6 patients aneurysms (only 2 pulmonary arterial aneurysms). Digestive involvement was present in 12 (9,1%) patients, with predominant colon involvement (8 cases). The most prescribed drugs were corticosteroids (85.6%) and colchicine (77.3%), followed by azathioprine (36.4%) and cyclosporine a (33.3%). Other prescribed drugs were thalidomide (6.1%), chlorambucil (6.9%), methotrexate (4.5%), anti-TNF-alpha therapies (infliximab 6.8% and adalimumab 2.3%), cyclophosphamide (3%), mycophenolate (3.8%), leflunomide (1.5%) and 22% received anticoagulation. Conclusion: Clinical features of our patients are similar to those of other European cohorts, although a high prevalence of organic involvement (ocular, neurological, vascular and joint) should be highlighted. References [1] Saadoun D, Wechsler B. Behcet’s disease. Orphanet J Rare Dis. 2012Apr12;7;20. doi: 10.1186/1750-1172-7-20. [2] Mendes D, et al. Behcet’s disease – a contemporary rewiew. J autoimmun. 2009May-Jun;32 (3-4): 178-88. doi: 10.1016/j.jaut.2009.02.011. [3] Leccese P, et al. Behcet’s sindrome in nonendemic regions. Curr Opin Rheumatol. 2017. doi: 10.1097/BOR.0000000000000349 Disclosure of interests: None declared

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 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

Objectives To analyse how the age at diagnosis modifies the phenotype of primary Sjogren syndrome (SS) Methods The Big Data Sjogren Project was formed in 2014 to take a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2019, the database included 11,420 patients from 24 countries of the 5 continents. Results Women (52.7 vs 54.6 yrs in men, p Patients with positive immunological markers had a younger diagnostic age, except for cryoglobulins (p Patients without systemic activity (ESSDAI score = 0) were diagnosed at an older age (55.5 vs 52.1 yrs in those with systemic activity, p Conclusion Age at diagnosis plays a key role in the glandular and systemic phenotype expressed by primary SjS patients at the time of diagnosis. Disclosure of Interests: Soledad Retamozo: None declared, Nihan Acar-Denizli: None declared, Wan Fai Ng: None declared, Ildiko Fanny Horvath: None declared, Astrid Rasmussen: None declared, Raphaele Seror Grant/research support from: Pfizer, Consultant for: Bristol-Myers Squibb, Pfizer, Amgen, Eli Lilly, Roche, Celgene, GlaxoSmithKline, MedImmune, Xiaomei Li: None declared, Chiara Baldini: None declared, Jacques-Eric Gottenberg Grant/research support from: Bristol-Myers Squibb, Grant/research support from: Bristol-Myers Squibb, Consultant for: Bristol-Myers Squibb, Lilly, Pfizer, Sanofi-Genzyme, UCB Pharma, Consultant for: Bristol-Myers Squibb, Eli Lilly, UCB, Sanofi-Genzyme, Pfizer, Pulukool Sandhya: None declared, Luca Quartuccio: None declared, Roberta Priori: None declared, Gabriela Hernandez-Molina: None declared, Berkan Armagan: None declared, Aike A. Kruize: None declared, Seung-Ki Kwok: None declared, Marika Kvarnstrom: None declared, Sonja Praprotnik: None declared, Damien Sene: None declared, Elena Bartoloni Bocci: None declared, Roser Solans-Laque: None declared, Maureen Rischmueller Consultant for: Abbvie, Bristol-Meyer-Squibb, Celgene, Glaxo Smith Kline, Hospira, Janssen Cilag, MSD, Novartis, Pfizer, Roche, Sanofi, UCB, Thomas Mandl: None declared, Yasunori Suzuki: None declared, David Isenberg: None declared, Valeria Valim: None declared, Agata Sebastian: None declared, Gunnel Nordmark: None declared, Hendrika Bootsma: None declared, Hideki Nakamura: None declared, Roberto Giacomelli Grant/research support from: Pfizer, Actelion, Speakers bureau: Actelion, Bristol-Myers Squibb, Merck Sharp & Dohme, Abbvie, Pfizer, Sobi, Roche, Valerie Devauchelle-Pensec Grant/research support from: Roche-Chugai, Speakers bureau: MSD, BMS, UCB, Roche, Benedikt Hofauer Consultant for: Consultant for Galvani Bioelectronics for the area of sleep disorders., Michele Bombardieri Grant/research support from: Celgene, Consultant for: Medimmune, Virginia Fernandes Moca Trevisani: None declared, Daniel Hammenfors: None declared, Sandra Pasoto: None declared, Tamer A Gheita: None declared, Fabiola Atzeni: None declared, Jacques Morel: None declared, Cristina Vollenveider: None declared, Sandra Consani-Fernandez: None declared, Xavier Mariette Grant/research support from: Servier, Consultant for: AstraZeneca, Bristol-Myers Squibb, GlaxoSmithKline, Janssen, Pfizer, UCB Pharma, Manuel Ramos-Casals: None declared, Pilar Brito-Zeron: None declared

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: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome, is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis, associated with asthma, nasal poliposis and rinosinusitis, in which eosinophils paly a key role. Eosinophil targeted therapies alone or associated to conventional treatment with corticosteroids and immunosuppressant drugs may be useful in patients with refractory disease or asthma difficult to treat. Objectives: To describe the efficacy and safety of eosinophil targeted therapies in patients with relapsing or refractory EGPA Methods: Retrospective study including all patients with EGPA included at the REVAS Registry, who were treated with omalizumab, mepolizumab or resilizumab. Complete response (CR) was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of ≤ 7.5 mg/day, and partial response (PR) when the prednisone dosage was ≥ 7.5 mg/day. Statistical analysis was performed using SSPS 20 package. Results: Seventeen patients (median age 49 years) received omalizumab (n=9) for a mean period of 53.4 (5-91) months, mepolizumab (n=7) for a mean period of 13.6 (5-16) months, or resilizumab (n=1) for a mean period of 7 months, for severe steroid-dependent asthma (94.1%) and/or sinonasal involvement (82.4%). ANCA were positive in 8 (47.1%) cases with MPO specificity in 7 cases. All patients were receiving corticosteroids with a mean dosage of 15 mg/day prior to eosinophil targeted therapies institution. Four (57.1%) patients treated with omalizumab achieved a CR, 1 a PR, and 2 had no improvement. The median dosage of prednisone 6 months after omalizumab initiation was 10 (2.5-10) mg/day and the median dosage after 12 months 5 (0-10) mg/day. The median number of exacerbations decreased from 4 over the 6 months previous to therapy startment to 2 in the following 12 months. Both patients refractory to omalizumab were treated with mepolizumab with CR at 6 months in 1 case. Treatment with mepolizumab was also started in 4 patients treated with omalizumab after a prolonged period of treatment (72.4 months), due to recurrence of asthma and/or sinusitis, and peripheral blood eosinophilia >10%. Three (75%) patients achieved a CR, and 1 patient experienced a major vasculitis relapse needing immunosuppressant drugs. Six (85.7%) patients initially treated with mepolizumab achieved a complete remission after 6-12 months of treatment. One patient achieved a PR. The median dosage of prednisone 6 months after mepolizumab initiation was 5 (3.1-6.8) mg/day and the median dosage after 12 months 3.5 (0-5) mg/day. The median number of exacerbations decreased from 2.5 over the 6 months previous to therapy startment to 1 in the following 12 months. The patient treated with resilizumab achieved a CR at 6 months of therapy. All 3 drugs were safe and well tolerated. Conclusion: The results of the present study suggest that eosinophil targeted therapies have a corticosteroid-sparing effect in EGPA patients with asthmatic and/or sinonasal manifestations, and that may be used sequentially in refractory cases. However, reducing the corticosteroid dosage may also increase the risk of severe EGPA flares. More data are needed to widely recommend this therapy in EGPA patients. References: [1] AntiIgE monoclonal antibody (omalizumab) in refractory and relapsing Eosinohilic granulomatosis with polyangiitis (Churg-Struss). Arthritis Rheum 2016; 68: 2274-82 Disclosure of Interests: None declared

Objectives To analyse the phenotype of patients with primary Sjogren syndrome (SjS) in whom a lymphoproliferative disease is diagnosed concomitantly. Methods By January 2019, The Big Data Sjogren Project included 11,420 consecutive patients with primary SjS recruited from 24 countries of the five continents. Results 117 (1%) patients were diagnosed with lymphoma and primary SjS synchronously. Age-gender adjusted multivariate analysis identified the following features associated with lymphoma (OR; CI95%): male gender (4.61; 2.88-7.18), White ethnicity (3.51; 1.78-7.91), abnormal oral tests (3.4; 1.38-10.88), positive biopsy (3.2; 1.3-10.17), positive RF (2.27; 1.48-3.53), hypocomplementemia (3.39; 2.06-5.54), and cryoglobulins (4.74; 2.57-8.38). Activity (score > 1) in the constitutional (2.97; 1.86-4.62), glandular (3.11; 2.1-4.57), cutaneous (2.17; 1.28-3.52), peripheral nerve (2.56; 1.4-4.41) and hematological (2.49; 1.64-3.75) ESSDAI domains was associated with lymphoma (frequencies summarized in the Figure). Conclusion Patients diagnosed concomitantly with primary SjS and lymphoma have a very specific, highly-active phenotype (men, White, severe oral involvement, cryoglobulinemic-related immunological markers, and high systemic activity). Disclosure of Interests: Soledad Retamozo: None declared, Nihan Acar-Denizli: None declared, Wan Fai Ng: None declared, Antonia Szanto: None declared, Astrid Rasmussen: None declared, Raphaele Seror Grant/research support from: Pfizer, Consultant for: Bristol-Myers Squibb, Pfizer, Amgen, Eli Lilly, Roche, Celgene, GlaxoSmithKline, MedImmune, Xiaomei Li: None declared, Chiara Baldini: None declared, Jacques-Eric Gottenberg Grant/research support from: Bristol-Myers Squibb, Grant/research support from: Bristol-Myers Squibb, Consultant for: Bristol-Myers Squibb, Lilly, Pfizer, Sanofi-Genzyme, UCB Pharma, Consultant for: Bristol-Myers Squibb, Eli Lilly, UCB, Sanofi-Genzyme, Pfizer, Pulukool Sandhya: None declared, Luca Quartuccio: None declared, Roberta Priori: None declared, Gabriela Hernandez-Molina: None declared, Berkan Armagan: None declared, Aike A. Kruize: None declared, Seung-Ki Kwok: None declared, Marika Kvarnstrom: None declared, Sonja Praprotnik: None declared, Damien Sene: None declared, Roser Solans-Laque: None declared, Maureen Rischmueller Consultant for: Abbvie, Bristol-Meyer-Squibb, Celgene, Glaxo Smith Kline, Hospira, Janssen Cilag, MSD, Novartis, Pfizer, Roche, Sanofi, UCB, Thomas Mandl: None declared, Yasunori Suzuki: None declared, David Isenberg: None declared, Valeria Valim: None declared, Agata Sebastian: None declared, Gunnel Nordmark: None declared, Hendrika Bootsma: None declared, Hideki Nakamura: None declared, Roberto Giacomelli Grant/research support from: Pfizer, Actelion, Speakers bureau: Actelion, Bristol-Myers Squibb, Merck Sharp & Dohme, Abbvie, Pfizer, Sobi, Roche, Valerie Devauchelle-Pensec Grant/research support from: Roche-Chugai, Speakers bureau: MSD, BMS, UCB, Roche, Benedikt Hofauer Consultant for: Consultant for Galvani Bioelectronics for the area of sleep disorders., Michele Bombardieri Grant/research support from: Celgene, Consultant for: Medimmune, Virginia Fernandes Moca Trevisani: None declared, Daniel Hammenfors: None declared, Sandra Pasoto: None declared, Tamer A Gheita: None declared, Fabiola Atzeni: None declared, Jacques Morel: None declared, Cristina Vollenveider: None declared, Sandra Consani-Fernandez: None declared, Xavier Mariette Grant/research support from: Servier, Consultant for: AstraZeneca, Bristol-Myers Squibb, GlaxoSmithKline, Janssen, Pfizer, UCB Pharma, Manuel Ramos-Casals: None declared, Pilar Brito-Zeron: None declared, Elena Bartoloni Bocci: None declared

Objective.To analyze the role that infections play on the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) outcome.Methods.A retrospective study of adult patients with AAV diagnosed in a tertiary center. Clinical features, laboratory findings, treatment, relapses, major infections, and outcome were evaluated.Results.Included were 132 patients [51 microscopic polyangiitis (MPA), 52 granulomatosis with polyangiitis (GPA), 29 eosinophilic GPA (EGPA)] with a mean followup of 140 (96–228) months. ANCA were positive in 85% of cases. A total of 300 major infections, mainly bacterial (85%), occurred in 60% patients during the followup. Lower respiratory tract (64%) and urinary tract infections (11%) were the most frequent, followed by bacteremia (10%). A total of 7.3% opportunistic infections were observed, most due to systemic mycosis. Up to 46% of all opportunistic infections took place in the first year of diagnosis, and 55% of them under cyclophosphamide (CYC) treatment. Bacterial infections were associated with Birmingham Vasculitis Activity Score (version 3) > 15 at the disease onset, a total cumulative CYC dose > 8.65 g, dialysis, and development of leukopenia during the followup. Leukopenia was the only factor independently related to opportunistic infections. Forty-four patients died, half from infection. Patients who had major infections had an increased mortality from any cause.Conclusion.Our results confirm that major infections are the main cause of death in patients with AAV.

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.