SAT0261 CHARACTERISTICS ASSOCIATED TO SCLERODERMAL RENAL CRISIS, AND INCIDENCE VARIATION OVER TIME IN THE RESCLE REGISTRY

Abstract

Background: Scleroderma Renal Crisis (SRC) is a serious complication of Systemic Sclerosis (SSc). Nowadays, it seems that there is a reduction in its prevalence and mortality1. Objectives: To evaluate the characteristics of patients with SRC in a large cohort of SSc pacients. To investigate predictors of SRC, and epi-demiologic differences over time. Methods: 1933 patients were collected in ongoing registry of Spanish SSc pacients-RESCLE. We did descriptive study and epidemiologic analysis. Results: Out 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. Conclusion: In 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.