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BackgroundScleroderma Renal Crisis (SRC) is a serious complication of Systemic Sclerosis (SSc). Nowadays, it seems that there is a reduction in its prevalence and mortality1.ObjectivesTo evaluate the characteristics of patients with SRC in a large cohort of SSc pacients. To investigate predictors of SRC, and epidemiologic differences over time.Methods1933 patients were collected in ongoing registry of Spanish SSc pacients – RESCLE. We did descriptive study and epidemiologic analysis.ResultsOut of 1933 SSc, 43 (2.2%) developed SRC. Univariate analysis showed significant differences of SRC vs. non-SRC cases: SSc subtypes: diffuse cutaneous SSc (dcSSc), 72% vs. 19%; limited cutaneous SSc (lcSSc), 26% vs. 61%. Demographics: Female gender, 77% vs. 89%; time from SSc onset to SSc diagnosis, 3.0±8.0 vs. 6.6±9.5 years; arterial hypertension (HT), 56% vs. 32%. 1st manifestation: Raynaud’s phenomenon (RP), 68% vs. 82%. Clinical manifestations: RP, 88% vs. 96%; digital ulcers, 70% vs. 38%; arthritis, 45% vs. 20%; myositis, 30% vs. 13%; joint contractures, 45% vs. 18%; intestinal involvement, 24% vs. 11%; malabsorption, 24% vs. 7%; interstitial lung disease 58% vs. 41%; pulmonary HT, 56% vs. 29%; pericardial effusion, 28% vs. 7.4%; pericarditis, 24% vs. 8.3%; ischemic cardiopathy, 31% vs. 12%; diastolic dysfunction, 67% vs. 34%. Capillaroscopy: active pattern 77% vs. 33%. Immunological data: anti-Topoisomerase I, 39% vs. 20%; anti-centromere, 15% vs. 49%; anti-RNApol III, 45% vs. 11%. Prognosis: Overall mortality, 56% vs. 18%; SSc-related mortality, 83% vs. 49%. Survival at 5, 10, 20, and 30 years was 73% vs. 96%, 56% vs. 92%, 28% vs. 80%, and 28% vs. 67%, respectively. Treatment: ACEI use, 35% vs. 14%, corticoid use, 51% vs. 25%. Multivariate analysis: dsSSc subtype, RR 22.68 (5.81-88.51) p<0.001; intestinal malabsorption, RR 7.35 (2.55-21.18) p<0.001, and active capillaroscopy pattern RR 7.26 (1.61-32.7) p<0.010. Prevalence of SRC (P) in dcSSc subtype 7.8%, and in lcSSc subtype 0.9%. P over decades: P-80’s, 3.8%; P-90’s, 2.7%; P-00’s: 2.3% and P-10’s: 0.9%, achieving statistical significance in the last decade RR: 0.33 (0.13-0.85) p=0.014.ConclusionIn RESCLE cohort, SRC predominated in dcSSc patients, and it was associated to intestinal malabsorption, and an active pattern by capillaroscopy. SRC showed a very poor prognosis. Finally, we evidenced a decreasing prevalence of SRC over time in our cohort.Reference[1] Turk M, et al. The Frequency of Scleroderma Renal Crisis over Time: A Metaanalysis. J Rheumatol 2016; 43: 1350.Disclosure of InterestsNone declared

Background:Anti-PM/Scl antibodies are associated to systemic sclerosis (SSc) but are not specific to SSc. The true prevalence of anti-PM/Scl antibodies in SSc is unknown, ranging from 2.5% to 12.5%. An association between anti-PM/Scl antibodies with muscular involvement, pulmonary fibrosis, calcinosis, and a relatively benign prognosis have been described.Objectives:To compare the clinical manifestations and prognosis of SSc patients according the presence of anti-PM/Scl antibodies in the cohort of RESCLE (Spanish Scleroderma Registry).Methods:From the Spanish Scleroderma Study Group database, we selected patients in whom anti-PM/Scl antibodies had been tested. We compared demographic features, clinical manifestations, laboratory characteristics, and survival data between patients according the anti-PM/Scl antibodies status.Results:72 out of 947 (7%) patients tested positive for anti-PM/Scl antibodies. As presenting SSc manifestations, patients with anti-PM/Scl antibodies had higher prevalence of puffy fingers (11% versus 2%; p=0.002) and arthralgias (11% versus 4%; p=0.03), and lower prevalence of Raynaud’s phenomenon (65% versus 82%, p=0.002). Regarding cumulative manifestations, myositis (51% versus 15%; p<0.001), arthritis (43% versus 22%; p=0.001), and interstitial lung disease (ILD) (60% versus 45%, p=0.014) were more prevalent in patients with anti-PM/Scl antibodies. In fact, those patients with anti-Pm/Scl antibodies presented with FVC (77.4% ± 23.1% versus 85.8% ± 23,1%; p=0.006) and more severe ILD defined as FVC <70% (41% versus 24%; p=0.004). Death rate was similar in patients with and without PM/Scl antibodies (18% versus 17%; p=0.871).We did not find differences in terms of death rate nor in the causes of death (SSc and non-SSc related) according to the anti-PM/Scl antibodies profile.The 5- and 10-years survival rates of patients with anti-PM/Scl antibodies were 91% and 82% respectively, without differences with those without these antibodies (93% and 85%, respectively).Conclusion:In Spanish SSc patients, the presence of anti-PM/Scl antibodies confer a distinctive clinical profile. However, anti-PM/Scl antibodies do not play a role in the prognosis of these patients.References:[1]Stochmal A, Czuwara J, Trojanowska M, Rudnicka L. Antinuclear antibodies in systemic sclerosis: an update. Clin Rev Allergy Immunol 2020;58(1):40-51. doi: 10.1007/s12016-018-8718-8.Acknowledgments:We gratefully acknowledge all investigators who are part of the RESCLE Registry. We also thank the RESCLE Registry Coordinating Centre, S&H Medical Science Service, for their quality control data, logistic and administrative support and Prof. Salvador Ortiz, Universidad Autónoma de Madrid and Statistical Advisor S&H Medical Science Service for the statistical analysis of the data presented in this paper.Disclosure of InterestsNerea Iniesta-Arandia: None declared, Gerard Espinosa Speakers bureau: Glaxo-Smith-Kline, Janssen, Boehringer, Rovi, Alfredo Guillen del Castillo: None declared, Carles Tolosa Consultant of: Actelion pharmaceuticals, GSK, MSD., Gema Maria Lledó: None declared, Dolores Colunga Argüelles Consultant of: Actelion pharmaceuticals, GSK, MSD., Cristina González-Echávarri: None declared, Luis Sáez-Comet: None declared, Norberto Ortego: None declared, Jose Antonio Vargas-Hitos: None declared, Manuel Rubio-Rivas: None declared, Mayka Freire: None declared, Juan José Rios: None declared, Monica Rodriguez-Carballeira: None declared, Luis Trapiella Martínez: None declared, Vicent Fonollosa Pla Speakers bureau: Actelion, Carmen Pilar Simeón-Aznar Consultant of: Actelion pharmaceuticals, GSK, MSD., on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS): None declared