RT Journal Article SR Electronic T1 Serologic Profile and Mortality Rates of Scleroderma Renal Crisis in Italy JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 1464 OP 1469 DO 10.3899/jrheum.080806 VO 36 IS 7 A1 VERONICA CODULLO A1 ILARIA CAVAZZANA A1 CLAUDIA BONINO A1 CLAUDIA ALPINI A1 LORENZO CAVAGNA A1 FRANCO COZZI A1 NICOLETTA DEL PAPA A1 FRANCO FRANCESCHINI A1 SERENA GUIDUCCI A1 GABRIELLA MOROZZI A1 AMELIA RUFFATTI A1 CLODOVEO FERRI A1 ROBERTO GIACOMELLI A1 MARCO MATUCCI-CERINIC A1 GABRIELE VALENTINI A1 CARLOMAURIZIO MONTECUCCO YR 2009 UL http://www.jrheum.org/content/36/7/1464.abstract AB 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.