Atypical Hemolytic‐Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment. Issue 1 (29th October 2018)

• Record Type: Journal Article
• Authors: Raina, Rupesh; Krishnappa, Vinod; Blaha, Taryn; Kann, Taylor; Hein, William; Burke, Linda; Bagga, Arvind
• Is Part Of: Therapeutic apheresis and dialysis. Volume 23:Issue 1(2019)
• Page Start: 4
• Page End: 21
• Publication Date: 2018-10-29
• Subjects: Atypical hemolytic uremic syndrome -- Complement regulatory protein mutation -- Eculizumab -- Renal impairment -- Thrombotic microangiopathy; Hemapheresis -- Periodicals; Dialysis -- Periodicals; Blood Component Removal -- Periodicals; Renal Dialysis -- Periodicals; Hémaphérèse -- Périodiques; Dialyse -- Périodiques; Sang -- Collecte et conservation -- Périodiques; 616
• DOI: 10.1111/1744-9987.12763
• Languages: English
• ISSNs: 1744-9979

Abstract

Abstract: 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.