Clinical Features of Anti-Factor H Autoantibody-Associated Hemolytic Uremic Syndrome

• Published in: Journal of the American Society of Nephrology Vol. 21; no. 12; pp. 2180 - 2187
• Main Authors: DRAGON-DUREY, Marie-Agnès, SIDHARTH KUMAR SETHI, LE QUINTREC, Moglie, NIAUDET, Patrick, LOIRAT, Chantal, HERMAN FRIDMAN, Wolf, FREMEAUX-BACCHI, Véronique, BAGGA, Arvind, BLANC, Caroline, BLOUIN, Jacques, RANCHIN, Bruno, ANDRE, Jean-Luc, TAKAGI, Nobuaki, HAE II CHEONG, HARI, Pankaj
• Format: Journal Article
• Language: English
• Published: Washington, DC American Society of Nephrology 01.12.2010
• Subjects: Adult; Age Factors; Autoantibodies - immunology; Biological and medical sciences; Child; Child, Preschool; Clinical Research; Cohort Studies; Complement Activation - immunology; Complement Factor H - immunology; Female; Follow-Up Studies; Hematologic and hematopoietic diseases; Hemolytic-Uremic Syndrome - diagnosis; Hemolytic-Uremic Syndrome - epidemiology; Hemolytic-Uremic Syndrome - immunology; Hemolytic-Uremic Syndrome - therapy; Humans; Immunoglobulins, Intravenous - administration & dosage; Infant; Kidney Transplantation; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Other diseases. Hematologic involvement in other diseases; Plasma Exchange - methods; Renal failure; Risk Assessment; Severity of Illness Index; Sex Factors; Stem Cell Transplantation - methods; Time Factors; Treatment Outcome; Kidney disease; Autoimmunity; Human; Thrombocytopenia; Nephrology; Factor H; Urinary system disease; Prognosis; Hemolytic uremic syndrome; Antibody; Acute; Autoantibody; Cardiovascular disease; Hemopathy; Thrombohemolytic microangiopathy; Urology; Vascular disease; Platelet; Hemolytic anemia; Treatment; Renal failure; Genetics; Diagnosis
• ISSN: 1046-6673; 1533-3450
• DOI: 10.1681/asn.2010030315

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy that associates, in 70% of cases, with genetic or acquired disorders leading to dysregulation of the alternative pathway of complement. Autoantibody directed against Factor H causes at least 6% to 10% of aHUS cases, but only a few clinical reports are available. Here, we describe the clinical, biologic, genetic features, treatment, and outcome of 45 patients who presented with aHUS associated with anti-FH autoantibody. We found that this form of aHUS primarily affects children between 9 and 13 years old but it also affects adults. It presents with a high frequency of gastrointestinal symptoms and with extrarenal complications and has a relapsing course. Activation of the alternative pathway of complement at the onset of disease portends a poor prognosis. Early specific treatment may lead to favorable outcomes. These data should improve the recognition and diagnosis of this form of aHUS and help identify patients at high risk of a poor outcome.