Open AccessComplement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report
Author(s) -
Torres Everardo Arias,
Chang Yongen,
Desai Sheetal,
Chang Ian,
Zuckerman Jonathan E.,
Burwick Richard,
Kalantar-Zadeh Kamyar,
Hanna Ramy M.
Publication year - 2021
Publication title -
case reports in nephrology and dialysis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.278
H-Index - 7
ISSN - 2296-9705
DOI - 10.1159/000512227
Subject(s) - single case
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.