Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab

• Published in: Hematology reports Vol. 8; no. 3; p. 6625
• Main Authors: Raufi, Alexander G, Scott, Shruti, Darwish, Omar, Harley, Kevin, Kahlon, Kanwarpal, Desai, Sheetal, Lu, Yuxin, Tran, Minh-Ha
• Format: Journal Article
• Language: English
• Published: Italy MDPI AG 30-09-2016; PAGEPress Scientific Publications, Pavia, Italy; PAGEPress Publications, Pavia, Italy
• Subjects: Atypical hemolytic uremic syndrome; Case Report; Complement inhibitors; Hemolytic anemia; Hemolytic uremic syndrome; Hypocomplementemia; Lupus; Lupus nephritis; Microangiopathic hemolytic anemia; Nephritis; Renal failure; Systemic lupus erythematosus; Thrombocytopenia; Thrombocytopenic purpura; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura; Atypical hemolytic uremic syndrome; Thrombotic microangiopathy; Lupus nephritis; Microangiopathic hemolytic anemia
• ISSN: 2038-8322; 2038-8330; 2038-8330
• DOI: 10.4081/hr.2016.6625

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.