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Review
. 2024 Dec;44(12):3135-3140.
doi: 10.1007/s00296-024-05689-z. Epub 2024 Aug 15.

Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review

Zeynep Toker Dincer  1 , Mevlut Tamer Dincer  2 , Gozde Sen  3 , Serdal Ugurlu  4 , Nurhan Seyahi  2 , Emire Seyahi  4
Affiliations
Review

Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review

Zeynep Toker Dincer et al. Rheumatol Int. 2024 Dec.

Abstract

Systemic sclerosis (SSc) poses significant challenges in clinical management, especially when complicated by scleroderma renal crisis (SRC), a rare but life-threatening manifestation. Here, we report a 41-year-old female patient with SSc who presented with SRC and concurrent thrombotic microangiopathy. Her condition persisted despite conventional treatments such as plasma exchange and renin-angiotensin-aldosterone system blockade. In particular, treatment with eculizumab, a C5 complement inhibitor, led to a rapid improvement in platelet count, reduction in lactate dehydrogenase levels, and complete recovery of renal function. Genetic testing revealed a variant of unknown significance in the thrombomodulin (THBD) gene, which is associated with the complement system. This case highlights the complex interplay between complement dysregulation and SRC, and highlights the promising role of eculizumab in refractory cases. Further investigation of complement involvement and the efficacy of eculizumab in SRC warrants attention to improving therapeutic outcomes in this challenging condition.

Keywords: Acute kidney injury; Complement system; Systemic sclerosis; Thrombotic microangiopathy.

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Conflict of interest statement

Declarations. Conflict of interest: The authors declare that they have no conflict of interest. Editing and AI disclaimer: This manuscript has not been prepared with the assistance of professional editing agencies or AI-based editing software. Ethics: Written informed consent was obtained from the patient for the publication of this case report.

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