Corticosteroids and the risk of scleroderma renal crisis: a systematic review

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, EMBASE, 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 definite cases of SRC, equivalent to a rate of 2%, were identified. In the subset of early diffuse patients, the rate of SRC was 4%. All 10 definite 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.