title: Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab.
abstract: Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura - a TMA classically associated with autoimmune depletion of ADAMTS-13 - aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Alexander G
authors: AG
authors: Raufi
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Shruti
authors: S
authors: Scott
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Omar
authors: O
authors: Darwish
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Kevin
authors: K
authors: Harley
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Kanwarpal
authors: K
authors: Kahlon
authors: Department of Internal Medicine, University of California , Irvine, CA, USA.
authors: Sheetal
authors: S
authors: Desai
authors: Department of Pathology and Laboratory Medicine, University of California , Irvine, CA, USA.
authors: Yuxin
authors: Y
authors: Lu
authors: Department of Pathology and Laboratory Medicine, University of California , Irvine, CA, USA.
authors: Minh-Ha
authors: MH
authors: Tran
keywords: Atypical hemolytic uremic syndrome; Lupus nephritis; Microangiopathic hemolytic anemia; Thrombotic microangiopathy