International Journal of Nephrology and Renovascular Disease (Sep 2019)
Optimal management of atypical hemolytic uremic disease: challenges and solutions
Abstract
Rupesh Raina1,2,*, Manpreet K Grewal2,*, Yeshwanter Radhakrishnan3, Vineeth Tatineni4, Meredith DeCoy5, Linda LG Burke5, Arvind Bagga6 1Department of Nephrology, Akron Children’s Hospital, Akron, OH, USA; 2Akron Nephrology Associates, Cleveland Clinic Akron General, Akron, OH, USA; 3Department of Internal Medicine, Cleveland Clinic Akron General Hospital, Akron, OH, USA; 4Department of Internal Medicine, Summa Health, Akron City Hospital, Akron, OH, USA; 5Atypical Hemolytic Uremic Syndrome Alliance, Cape Elizabeth, ME, USA; 6Division of Paediatric Nephrology, All India Institute of Medical Sciences, New Delhi, India*These authors contributed equally to this workCorrespondence: Rupesh RainaDepartment of Nephrology, Cleveland Clinic Akron General, 224 W. Exchange St., Akron, OH 44302, USATel +1 330 436 3150Email [email protected]: Atypical hemolytic uremic syndrome (aHUS) is a chronic life threatening condition that arises from genetic abnormalities resulting in uncontrolled complement amplifying activity. The introduction of eculizumab, the humanized monoclonal antibody, has brought about a paradigm shift in the management of aHUS. However, there are many knowledge gaps, diagnostic issues, access and cost issues, and patient or physician challenges associated with the use of this agent. Limited data on the natural history of aHUS along with the underlying genetic mutations make it difficult to predict the relapses and thereby raising concerns about the appropriate duration and monitoring of treatment. In this review, we discuss the safety and efficacy of eculizumab in patients with aHUS and its associated challenges.Keywords: atypical hemolytic uremic syndrome, eculizumab, challenges, thrombotic microangiopathy
