Atypical hemolytic uremic syndrome and complement blockade: established and emerging uses of complement inhibition.
PMID 30865166 · Current opinion in nephrology and hypertension · 2019 · DOI 10.1097/MNH.0000000000000499
Authors: Ramy M Hanna; Marina Barsoum; Andrae Vandross; Ira Kurtz; Richard Burwick
Abstract
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. 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. 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.
Atypical Hemolytic Uremic Syndrome; Complement Activation; Complement Inactivating Agents; Humans
Cite
Ramy M Hanna; Marina Barsoum; Andrae Vandross; Ira Kurtz; Richard Burwick. Atypical hemolytic uremic syndrome and complement blockade: established and emerging uses of complement inhibition. Current opinion in nephrology and hypertension (2019). PMID 30865166. DOI 10.1097/MNH.0000000000000499.