%0 Journal Article %A VERONICA CODULLO %A ILARIA CAVAZZANA %A CLAUDIA BONINO %A CLAUDIA ALPINI %A LORENZO CAVAGNA %A FRANCO COZZI %A NICOLETTA DEL PAPA %A FRANCO FRANCESCHINI %A SERENA GUIDUCCI %A GABRIELLA MOROZZI %A AMELIA RUFFATTI %A CLODOVEO FERRI %A ROBERTO GIACOMELLI %A MARCO MATUCCI-CERINIC %A GABRIELE VALENTINI %A CARLOMAURIZIO MONTECUCCO %T Serologic Profile and Mortality Rates of Scleroderma Renal Crisis in Italy %D 2009 %R 10.3899/jrheum.080806 %J The Journal of Rheumatology %P 1464-1469 %V 36 %N 7 %X Objective. To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc). Methods. A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit III, and immunoprecipitation (IP) were performed. Results. Forty-six cases (38 female; 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years ± 13.2 and 55.4 years ± 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%), anti-RNAP I–III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I–III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being lower in the anti-topo I than in the anti-RNAP I–III group (p = 0.034). Conclusion. SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I–III in our patients with SRC and was associated with delayed onset and high mortality rates. %U https://www.jrheum.org/content/jrheum/36/7/1464.full.pdf