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Rheumatol Int (2012) 32:645653 DOI 10.1007/s00296-010-1697-6
ORIGINAL ARTICLE
Corticosteroids and the risk of scleroderma renal crisis: a systematic review
Gerald Trang Russell Steele Murray Baron
Marie Hudson
Received: 15 July 2010 / Accepted: 21 November 2010 / Published online: 4 December 2010 Springer-Verlag 2010
Abstract Scleroderma renal crisis (SRC) has been associated with the use of corticosteroids (CS) in retrospective studies. Using an evidence-based approach, we undertook a systematic review of the literature to identify prospective studies in which scleroderma patients were administered CS to ascertain the risk of SRC in those patients. A comprehensive search was conducted using Medline, EM-BASE, the Cochrane Library, and Web of Science. All original prospective clinical studies were eligible if they enrolled SSc patients newly treated with CS. Selected studies were reviewed, and data extraction was systematically performed for the dose and duration of the CS intervention as well as the occurrence of SRC. Twenty-six studies with a total of 500 SSc patients commencing new CS therapy were included in the systematic review. Ten denite cases of SRC, equivalent to a rate of 2%, were identied. In the subset of early diffuse patients, the rate of SRC was 4%. All 10 denite cases of SRC occurred in patients who received medium- to high-dose CS therapy. Seven cases occurred in the setting of stem cell transplant. CS are associated with SRC, although this may be due to
confounding by disease severity and/or co-intervention. Great caution must continue to be exerted when initiating such therapy, especially in high doses and in the early diffuse subset of SSc patients.
Keywords Systemic sclerosis Scleroderma renal crisis
Corticosteroids Systematic review
Introduction
Systemic sclerosis (SSc) is a connective tissue disease of uncertain aetiology characterized by the pathophysiologic triad of vascular damage, brosis of the skin and internal organs, and autoimmunity and inammation [1]. Scleroderma renal crisis (SRC) is one of its most feared complications. It is characterized by acute renal failure usually accompanied by malignant hypertension [2]. Although the advent of ACE inhibitors has dramatically improved the outcome from SRC [3], mortality remains high, climbing up to 50% within a year in one study of early diffuse systemic sclerosis (dcSSc) patients [4]. A signicant number of patients who survive require short- and/or long-term dialysis,...