Complement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report

• Main Authors: Everardo Arias Torres, Yongen Chang, Sheetal Desai, Ian Chang, Jonathan E. Zuckerman, Richard Burwick, Kamyar Kalantar-Zadeh, Ramy M. Hanna
• Format: Article
• Language: English
• Published: Karger Publishers 2021-03-01
• Series: Case Reports in Nephrology and Dialysis
• Subjects: thrombotic microangiopathy; systemic lupus erythematosus; complement-mediated thrombotic microangiopathy; glomerular disease; pregnancy; atypical hemolytic uremic syndrome
• Online Access: https://www.karger.com/Article/FullText/512227

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. We present an important case of a pregnant 23-year-old Hispanic female who presented in mid-gestation (21 weeks) with an initial diagnosis of systemic lupus erythematosus (SLE) complicated by aHUS. She met clinical criteria for aHUS on presentation and was found to have a pathogenic CFHR1–3 homozygous deletion. She has been treated with intravenous and oral steroids, cyclophosphamide, subsequently also with plasma exchange, and finally with eculizumab with partial improvement in renal function. This case adds to the emerging literature showing that SLE and aHUS (or complement-mediated TMA) can be successfully treated with C5 blockade.