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Atypical hemolytic uremic syndrome and complement blockade

established and emerging uses of complement inhibition

Hanna, Ramy M.a; Barsoum, Marinaa; Vandross, Andraeb; Kurtz, Iraa,c; Burwick, Richardd

Current Opinion in Nephrology and Hypertension: May 2019 - Volume 28 - Issue 3 - p 278–287
doi: 10.1097/MNH.0000000000000499

Purpose of review Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders.

Recent findings In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficiency. In the purest sense, aHUS is a genetic condition that is activated (or unmasked) by an environmental exposure. However, it is now evident that complement activation is a feature of many diseases. Variants in complement regulatory genes predispose to microangiopathic hemolysis in many rheumatologic, oncologic, and drug-induced vascular, obstetric, peritransplant, and infectious syndromes.

Summary Many ‘hemolysis syndromes’ overlap clinically with aHUS, and we review the literature on the treatment of these conditions with complement inhibition. New reports on the treatment of C3 glomerulopathy, Shiga toxin-related classic hemolytic uremic syndrome, and medication-related thrombotic microangiopathy will be reviewed as well.

aDivision of Nephrology

bDivision of Hematology Oncology, Department of Medicine, David Geffen School of Medicine

cBrain Research Institute, UCLA

dDepartment of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Cedars Sinai Medical Center, Los Angeles, California, USA

Correspondence to Ramy M. Hanna, MD, Division of Nephrology, Room 7-155 Factor Bldg, 700 Tiverton Ave, Los Angeles CA 90095, USA. Tel: +1 310 206 6741; e-mail:

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