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.