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The aim of this study was to evaluate the frequency of systemic treatment in a cohort of sarcoidosis patients and identify presenting clinical features as predictive factors of the need for systemic immunosuppressive therapy.Retrospective study of 342 patients diagnosed and followed-up from January 1999 to December 2019 in a University Hospital in Northern Spain. The diagnosis of sarcoidosis was established according to ATS/ERS/WASOG criteria. A comparative analysis was performed between treated and untreated patients. Predictive factors of treatment prescription according to initial clinical manifestations were identified (multivariate analysis).Mean age at diagnosis was 47.7±15.1 years, with a slight female predominance (51.8%) and Caucasian majority (94.2%). The main clinical manifestation was thoracic involvement (88.3%). Extrathoracic manifestations were detected in 68.4% cases, mainly cutaneous (34.2%), articular (27.8%) and ocular (17.8%). A total of 207 (60.5%) patients required systemic treatment. Glucocorticoid therapy was the most widely used (60.5%). Conventional immunosuppressive therapy in 25.4%, more frequently MTX (21.9%). Biologic therapy was prescribed in 12.9%, especially adalimumab (9.1%). Male gender (OR: 1.65; 95%CI: 1.06-2.56), intrathoracic (OR: 2.41; 95%CI: 1.22-4.76), ocular (OR: 4.14; 95%CI: 2.01-8.52), parotid (OR: 1.60; 95%CI: 1.39-1.94), neurological (OR: 5.00; 95%CI: 1.68-14.84), and renal (OR: 1.59; 95%CI: 1.38-1.94) involvement were identified as risk factors associated with the need of systemic treatment.Most patients (60.5%) of sarcoidosis in our series required systemic therapy. An association between certain characteristics at initial presentation (male gender, lung, ocular, parotid, neurological and renal involvement) and the need of systemic treatment was identified.

Background: COVID19 may present different degrees of severity. Viral infections in patients with rheumatic inflammatory diseases (R-IMID) tend to present more severe disease. However, data comparing the severity of the disease between R-IMID and the general population are scarce.Objectives: To compare predisposing factors, clinical, serological features, and severity of COVID-19 infection in patients with and without R-IMID.Methods: Case-control study in a single University Hospital. We included all consecutive patients with a diagnosis of an R-IMID and COVID-19 infection up to March 31st, 2021. This cohort was compared with patients without R-IMID and not receiving immunosuppressive therapy matched for sex and age (± 5 years). Confirmed infection was defined if the patient had a positive nasopharyngeal swab for SARS-CoV-2. COVID-19 severity was divided into mild, moderate, severe and critical according to the United States National Institute of Health (NIH) COVID-19 guidelines.Results: We included 274 R-IMID patients (185 women/89 men), mean age 59.1± 18 years. More frequent R-IMID were: Axial spondyloarthritis/ Psoriatic arthritis (32.5%), Rheumatoid arthritis (28.8%), Polymyalgia Rheumatica (8%) and Systemic Lupus Erythematosus (8%). Hypertension and dyslipidemia were more frequent in patients with R-IMID. In the R-IMID group, dyspnea was more frequent. Although most of the cases were mild, critical cases and deaths were more frequent in R-IMID. When adjusted by comorbidities, no statistical differences were observed.Conclusion: R-IMID have a very similar clinical presentation when compared to the general population. There is a trend to an increased severity of the disease in patients with R-IMID.

Tocilizumab (TCZ) is the only biologic therapy approved for giant cell arteritis (GCA). There is general agreement on the initial/maintenance dose, duration of TCZ therapy is not well established. In GiACTA trial, after one year on TCZ, most patients had GCA relapse after withdrawal. The aim of this study is to assess the effectiveness and safety of TCZ therapy optimisation in a large unselected series of patients with GCA in a clinical practice scenario.Multicentre study on 471 GCA patients treated with TCZ. Once prolonged remission was achieved (n=231) and based on a decision between patient and physician, TCZ was optimised (n=125). We compared optimised (TCZOPT) and not optimised (TCZNON-OPT) groups. Prolonged remission defined as normalisation of clinical and laboratory data for 6 months. Optimisation was carried out by decreasing TCZ dose and/or increasing dosing interval.We evaluated 231 GCA patients on TCZ in prolonged remission. At TCZ onset, no differences in demographic, clinical, or laboratory data were observed. First TCZ optimisation was performed after a median follow-up of 12[6-17] months. Intravenous TCZ was optimised from 8 to 4mg/kg/4weeks in 44% patients, while subcutaneous TCZ was optimised from 162mg/w to 162mg/every-other-week in 65% cases. At the end of follow-up, prolonged remission (78.2% vs. 84.2%; p=0.29) and relapses (5.6% vs. 10.4%, p=0.177) were similar in TCZOPT vs. TCZNON-OPT. Severe infections were more frequent in TCZNON-OPT (12.9% vs. 6.6%; p=0.009).TCZ optimisation may be done once complete remission is achieved by reducing dose or increasing dosing interval. This seems to be effective, safe and cost-effective therapeutic scheme.

Objective Rapid control of intraocular inflammation in non-infectious uveitis (NIU) is mandatory to avoid irreversible structural and functional damage. In this study, we assessed the efficacy and safety of intravenous methylprednisolone (IVMP) pulses in the treatment of NIU. Methods A retrospective case series of 112 patients who received IVMP for the treatment of NIU, either isolated or associated with different underlying diseases, was studied. Intraocular inflammation (anterior chamber cells and vitritis) was the primary outcome measure. Secondary outcome measures were macular thickness and best corrected visual acuity (BCVA). Patients were assessed at baseline visit, and at days 2-5, 7, 15 and 30 after initiation of IVMP pulse therapy. Results A total of 112 patients (mean age 42 +/- 14.5 yrs) were assessed. An underlying immune-mediated disease was diagnosed in 73 patients. Inflammatory ocular patterns were panuveitis (n=68), posterior uveitis (n=30), anterior uveitis (AU) (n=12), and intermediate uveitis (n=2). Additionally, patients presented cystoid macular oedema (CME) (n=50), retinal vasculitis (n=37), and exudative retinal detachment (n=31). Therapies used before IVMP included intraocular glucocorticoids (n=4), high-dose oral systemic glucocorticoids (n=77), and conventional (n=107) or biologic (n=40) immunosuppressive drugs. IVMP dose ranged from 80 to 1,000 mg/day for 3-5 consecutive days. Improvement was observed in AU, vitritis, BCVA, CME, and retinal vasculitis. At first month evaluation, total remission was achieved in 19 patients. Side effects of IVMP were respiratory infections (n=3), uncontrolled hyperglycaemia (n=1), herpes zoster (n=1), and oral candidiasis (n=1). Conclusion IVMP pulse therapy was effective and safe, and achieved rapid control of NIU.

Lupus nephritis (LN) is a severe complication of systemic lupus erythematosus (SLE). Unfortunately, 10-20% of patients with LN develop end-stage renal disease (ESRD), and renal transplantation may be a therapeutic option. However, concerns about LN recurrence after transplant have been reported. We aimed to assess long-term post-transplant graft and patient survival in LN compared to patients with non-autoimmune nephropathy (polycystic kidney disease - PCKD).We carried out a single-centre retrospective study of all patients who underwent renal transplantation due to LN in a referral unit between 1980 and 2018. This cohort was compared with a group of PCKD patients. The main outcome variables were graft and patient survival for up to 20 years, and the time-course of serum creatinine and proteinuria in the first 5 years after transplantation. Cumulative survival rates were estimated by the Kaplan-Meier method and compared using the log-rank test.We included 53 patients: LN group (n=21) and PCKD group (n=32). Baseline clinical characteristics were similar in both groups, except age at transplantation (39.8±11.3 years in the LN group and 46.6±5.0 years in the PCKD group; p=0.004). No significant differences were found regarding graft (p=0.59) or patient survival (p=0.087) at 20 years of follow-up.Despite concerns about LN recurrence after renal transplantation, this study shows that this procedure might be a safe alternative therapy for ESRD related to SLE and may provide long-term survival.

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

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

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

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

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

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

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

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

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

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

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

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

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

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