title: Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients.
abstract: OBJECTIVE
  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) micromol/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.
  
  
  CONCLUSIONS
  SRC remains associated with severe morbidity and mortality. CS might increase the risk of developing SRC. Further studies are needed to confirm these results.
authors.affiliation: Internal Medicine Department and Reference Center for Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, and UPRES EA 4058, Paris-Descartes University, Faculty of Medicine, France.
authors.forename: L
authors.initials: L
authors.lastname: Teixeira
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
authors.forename: A
authors.initials: A
authors.lastname: Mahr
authors.forename: A
authors.initials: A
authors.lastname: Berezné
authors.forename: C
authors.initials: C
authors.lastname: Agard
authors.forename: M
authors.initials: M
authors.lastname: Mehrenberger
authors.forename: L-H
authors.initials: LH
authors.lastname: Noël
authors.forename: P
authors.initials: P
authors.lastname: Trolliet
authors.forename: C
authors.initials: C
authors.lastname: Frances
authors.forename: J
authors.initials: J
authors.lastname: Cabane
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000328:Adult; D000368:Aged; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000009:adverse effects / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005602:France; D005938:Glucocorticoids / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D006435:Renal Dialysis; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality* / Q000628:therapy; D015996:Survival Rate
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D005938:Glucocorticoids