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Published on: June 8, 2022
Scleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy
Prochore Kamgang Semeu1, Maxime Taghavi2, Caroline Geers3
1Division of Nephrology, Department of Internal Medicine, ULB-VUB Center for Hereditary Metabolic Diseases, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium.
Abstract:
Scleroderma renal crisis is a severe complication of systemic sclerosis with a poor prognosis. Therefore, identifying precipitating factors is essential. Among known risk factors, only few are reversible. On the contrary, anti-C5 therapy appears effective, at least in some cases. We describe a 59-year-old man with diffuse cutaneous systemic sclerosis who developed life-threatening scleroderma renal crisis following ibuprofen administration. Despite aggressive management, he did not improve. Renal biopsy have displayed features of thrombotic microangiopathy but no complement deposition. We then discuss the pathomechanism of scleroderma renal crisis that could drive eculizumab treatment since some renal biopsies exhibit complement deposits and others do not.
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