title: Serologic profile and mortality rates of scleroderma renal crisis in Italy.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Rheumatology, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, 27100, Italy.
authors.forename: Veronica
authors.initials: V
authors.lastname: Codullo
authors.forename: Ilaria
authors.initials: I
authors.lastname: Cavazzana
authors.forename: Claudia
authors.initials: C
authors.lastname: Bonino
authors.forename: Claudia
authors.initials: C
authors.lastname: Alpini
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cavagna
authors.forename: Franco
authors.initials: F
authors.lastname: Cozzi
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Del Papa
authors.forename: Franco
authors.initials: F
authors.lastname: Franceschini
authors.forename: Serena
authors.initials: S
authors.lastname: Guiducci
authors.forename: Gabriella
authors.initials: G
authors.lastname: Morozzi
authors.forename: Amelia
authors.initials: A
authors.lastname: Ruffatti
authors.forename: Clodoveo
authors.initials: C
authors.lastname: Ferri
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.forename: Carlomaurizio
authors.initials: C
authors.lastname: Montecucco
mesh_terms: D000328:Adult; D000368:Aged; D000906:Antibodies / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D005260:Female; D006801:Humans; D007558:Italy; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000209:etiology / Q000276:immunology* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012318:RNA Polymerase I / Q000276:immunology; D012319:RNA Polymerase II / Q000276:immunology; D012320:RNA Polymerase III / Q000276:immunology; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology* / Q000401:mortality*
chemical_list: D000906:Antibodies; D000974:Antibodies, Antinuclear; D012319:RNA Polymerase II; D012318:RNA Polymerase I; D012320:RNA Polymerase III; D004264:DNA Topoisomerases, Type I