Scleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy

Author:

Kamgang Semeu Prochore1ORCID,Taghavi Maxime2ORCID,Geers Caroline3,Mouthon Luc4,Barreto Gutierrez Leonel5,Stordeur Patrick6

Affiliation:

1. Division of Nephrology, Department of Internal Medicine, ULB-VUB Center for Hereditary Metabolic Diseases, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium

2. Division of Nephrology, Department of Internal Medicine, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium

3. Department of Pathology, Brugmann University Hospital and UZ Brussel, Vrije Universiteit Brussel, Brussels, Belgium

4. Department of Internal Medicine and Reference Center for Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Faculty of Medicine, Paris-Descartes University, Paris, France

5. Department of Intensive Care, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium

6. National Reference Center for the Complement System, Laboratory of Immunology, LHUB-ULB, 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.

Publisher

SAGE Publications

Subject

Immunology,Rheumatology,Immunology and Allergy

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1. Ibuprofen;Reactions Weekly;2023-09-23

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