Document Detail


Serologic profile and mortality rates of scleroderma renal crisis in Italy.
MedLine Citation:
PMID:  19487262     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Veronica Codullo; Ilaria Cavazzana; Claudia Bonino; Claudia Alpini; Lorenzo Cavagna; Franco Cozzi; Nicoletta Del Papa; Franco Franceschini; Serena Guiducci; Gabriella Morozzi; Amelia Ruffatti; Clodoveo Ferri; Roberto Giacomelli; Marco Matucci-Cerinic; Gabriele Valentini; Carlomaurizio Montecucco
Publication Detail:
Type:  Journal Article; Multicenter Study     Date:  2009-06-01
Journal Detail:
Title:  The Journal of rheumatology     Volume:  36     ISSN:  0315-162X     ISO Abbreviation:  J. Rheumatol.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-01     Completed Date:  2009-09-03     Revised Date:  2010-05-04    
Medline Journal Info:
Nlm Unique ID:  7501984     Medline TA:  J Rheumatol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  1464-9     Citation Subset:  IM    
Affiliation:
Division of Rheumatology, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, 27100, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Antibodies / blood
Antibodies, Antinuclear / blood
DNA Topoisomerases, Type I / immunology
Female
Humans
Italy
Kaplan-Meiers Estimate
Kidney Diseases / etiology,  immunology*,  mortality*
Male
Middle Aged
Prognosis
RNA Polymerase I / immunology
RNA Polymerase II / immunology
RNA Polymerase III / immunology
Retrospective Studies
Scleroderma, Systemic / complications,  immunology*,  mortality*
Chemical
Reg. No./Substance:
0/Antibodies; 0/Antibodies, Antinuclear; EC 2.7.7.-/RNA Polymerase I; EC 2.7.7.-/RNA Polymerase II; EC 2.7.7.-/RNA Polymerase III; EC 5.99.1.2/DNA Topoisomerases, Type I
Comments/Corrections
Comment In:
J Rheumatol. 2010 May;37(5):1068; author reply 1069   [PMID:  20439528 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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