Kamgang Semeu Prochore, Taghavi Maxime, Geers Caroline, Mouthon Luc, Barreto Gutierrez Leonel, Stordeur Patrick
Division of Nephrology, Department of Internal Medicine, ULB-VUB Center for Hereditary Metabolic Diseases, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium.
Division of Nephrology, Department of Internal Medicine, Brugmann University Hospital, Université Libre de Bruxelles, Brussels, Belgium.
J Scleroderma Relat Disord. 2023 Oct;8(3):NP6-NP8. doi: 10.1177/23971983231163663. Epub 2023 Apr 4.
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.
硬皮病肾危象是系统性硬化症的一种严重并发症，预后较差。因此，识别诱发因素至关重要。在已知的风险因素中，只有少数是可逆的。相反，抗C5治疗似乎是有效的，至少在某些情况下是这样。我们描述了一名59岁的弥漫性皮肤系统性硬化症男性患者，在服用布洛芬后发生了危及生命的硬皮病肾危象。尽管进行了积极的治疗，他的病情仍未改善。肾活检显示有血栓性微血管病的特征，但无补体沉积。然后我们讨论了硬皮病肾危象的发病机制，由于一些肾活检显示有补体沉积而另一些则没有，这可能促使使用依库珠单抗进行治疗。
