title: Prognosis of scleroderma renal crisis: a long-term observational study.
abstract: BACKGROUND
  Despite the efficacy of angiotensin-converting enzyme (ACE) inhibitors, the prognosis of patients with Scleroderma renal crisis (SRC) is characterized by a high rate of mortality and progression into end-stage renal disease (ESRD). Our aim was to evaluate the prognosis of SRC in our cohort of scleroderma patients.
  
  
  METHODS
  We reviewed clinical charts of all our patients who developed SRC from 1980 to 2005. Outcome measures were ESRD, patients' survival and SRC-related mortality. ESRD was defined as the need for chronic dialysis; survival was calculated from the time of SRC occurrence by Kaplan-Meier method. All patients were treated with ACE inhibitors and 10 patients were also treated with plasma exchange (PEx). Indications to PEx were concomitant micro-angiopathic haemolytic anaemia or intolerance to high doses of ACE inhibitors. SPSS package was used for calculation.
  
  
  RESULTS
  Of 606 patients, affected with systemic sclerosis, who came at our observation during the aforementioned interval, 20 (3.3%) developed SRC. One year after SRC onset, 55% of patients developed ESRD. The survival rate was 70% at 1 year and 50% at 5 years; the mortality rate related to SRC was 35%. Notably, in the subgroup of patients treated with PEx, 20% developed ESRD; the survival rate was 90% at 1 year and 70% at 5 years; the mortality rate related to SRC was 10%.
  
  
  CONCLUSIONS
  Short-term prognosis of SRC has improved, but long-term prognosis remains disappointing. PEx in addition to ACE inhibitors seems to be a therapeutic option in patients with SRC who develop micro-angiopathy or are intolerant to high doses of ACE inhibitors.
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Cozzi
authors.forename: Piero
authors.initials: P
authors.lastname: Marson
authors.forename: Silvia
authors.initials: S
authors.lastname: Cardarelli
authors.forename: Maria
authors.initials: M
authors.lastname: Favaro
authors.forename: Tiziana
authors.initials: T
authors.lastname: Tison
authors.forename: Marta
authors.initials: M
authors.lastname: Tonello
authors.forename: Erika
authors.initials: E
authors.lastname: Pigatto
authors.forename: Giustina
authors.initials: G
authors.lastname: De Silvestro
authors.forename: Leonardo
authors.initials: L
authors.lastname: Punzi
authors.forename: Andrea
authors.initials: A
authors.lastname: Doria
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*; D013997:Time Factors