Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients. Issue 1 (8th June 2007)

• Record Type: Journal Article
• Authors: Teixeira, L; Mouthon, L; Mahr, A; Berezné, A; Agard, C; Mehrenberger, M; Noël, L-H; Trolliet, P; Frances, C; Cabane, J; Guillevin, L
• Is Part Of: Annals of the rheumatic diseases. Volume 67:Issue 1(2008)
• Page Start: 110
• Page End: 116
• Publication Date: 2007-06-08
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2006.066985
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: To describe presentation and outcome of patients with scleroderma renal crisis (SRC). Methods: SRC was defined as rapidly progressive oliguric renal insufficiency and/or rapidly progressive arterial hypertension occurring during the course of systemic sclerosis (SSc). Chronic dialysis-free survival was analysed using multivariate Cox proportional hazards regression models. The risk for developing SRC associated with corticosteroid (CS) exposure during the preceding 1- or 3-month periods was analysed according to a case–crossover design. Results: A total of 50 SSc patients aged 53.3 (14.5) (mean (SD)) years were included in the study. SRC occurred between 1979 and 2003, after a mean (SD) disease duration of 27.7 (49.1) months. A total of 43 (86%) patients had diffuse SSc, 5 (10%) had limited cutaneous SSc and 2 (4%) had SSc sine scleroderma. At the time of SRC, 10 (20%) patients were taking angiotensin converting enzyme inhibitors, and mean creatininaemia was 468 (293) μmol/l. A total of 28 (56%) patients required haemodialysis. In all, 11 patients underwent a renal biopsy, all of them had specific vascular lesions of SRC. Multivariate analyses retained age >53 years and normal blood pressure as independent predictors of decreased dialysis-free survival. Exposure to CS prior to SRC was identified in 30 (60%) patients. The odds ratios for developing SRC associated with CS exposure during the preceding 1- or 3-month periods were 24.1 (95% CI 3.0–193.8) and 17.4 (95% CI 2.1–144.0), respectively. Conclusion: SRC remains associated with severe morbidity and mortality. CS might increase the risk of developing SRC. Further studies are needed to confirm these results.