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

Everardo Arias Torres; Yongen Chang; Sheetal Desai; Ian Chang; Jonathan E Zuckerman; Richard Burwick; Kamyar Kalantar-Zadeh; Ramy M Hanna

doi:10.1159/000512227

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

Case Rep Nephrol Dial. 2021 Mar 31;11(1):95-102. doi: 10.1159/000512227. eCollection 2021 Jan-Apr.

Authors

Everardo Arias Torres¹, Yongen Chang², Sheetal Desai³, Ian Chang³, Jonathan E Zuckerman⁴, Richard Burwick⁵, Kamyar Kalantar-Zadeh², Ramy M Hanna²

Affiliations

¹ Department of Medicine, University of California, Irvine, California, USA.
² Division of Nephrology, Hypertension and Kidney Transplantation, Department of Medicine, University of California, Irvine, California, USA.
³ Division of Rheumatology, Department of Medicine, University of California, Irvine, California, USA.
⁴ Department of Pathology and Laboratory Medicine, University of California, Los Angeles, California, USA.
⁵ Department of Obstetrics and Gynecology, Maternal-Fetal Medicine, Cedars Sinai Medical Center, Los Angeles, California, USA.

Abstract

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.

Keywords: Atypical hemolytic uremic syndrome; Complement-mediated thrombotic microangiopathy; Glomerular disease; Pregnancy; Systemic lupus erythematosus; Thrombotic microangiopathy.

Publication types

Case Reports