title: Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls.
abstract: OBJECTIVE
  Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.
  
  
  METHODS
  Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls.
  
  
  RESULTS
  Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.
  
  
  CONCLUSIONS
  Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.
authors.affiliation: Department of Internal Medicine, Hôpital Cochin, Paris Cedex 14, France. loic.guillevin@cch.aphp.fr
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Alice
authors.initials: A
authors.lastname: Bérezné
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.forename: Luis
authors.initials: L
authors.lastname: Teixeira
authors.forename: Jacques
authors.initials: J
authors.lastname: Pourrat
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Christian
authors.initials: C
authors.lastname: Agard
authors.forename: Jean
authors.initials: J
authors.lastname: Cabane
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.forename: Jean-Robert
authors.initials: JR
authors.lastname: Harle
authors.forename: Isabelle
authors.initials: I
authors.lastname: Deleveaux
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D000328:Adult; D000368:Aged; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D016022:Case-Control Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*; D011379:Prognosis; D006435:Renal Dialysis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D005938:Glucocorticoids; D011241:Prednisone