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Atypical Hemolytic‐Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment
Author(s) -
Raina Rupesh,
Krishnappa Vinod,
Blaha Taryn,
Kann Taylor,
Hein William,
Burke Linda,
Bagga Arvind
Publication year - 2019
Publication title -
therapeutic apheresis and dialysis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.415
H-Index - 53
eISSN - 1744-9987
pISSN - 1744-9979
DOI - 10.1111/1744-9987.12763
Subject(s) - eculizumab , atypical hemolytic uremic syndrome , thrombotic microangiopathy , medicine , microangiopathic hemolytic anemia , complement system , pathophysiology , alternative complement pathway , immunology , hemolytic anemia , abnormality , autoantibody , thrombotic thrombocytopenic purpura , disease , antibody , platelet , psychiatry
Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi‐organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over‐activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy‐associated aHUS, and aHUS in renal transplant patients.