Extra-renal manifestations of atypical hemolytic uremic syndrome

Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease resulting from abnormal alternative complement activation with a wide range of clinical presentations. Extra-renal manifestations of aHUS can involve many organ systems, including the peripheral and central nervous, gastrointestinal, cardiovascular, integumentary, pulmonary, as well as the eye. While some of these extra-renal manifestations occur in the acute phase of aHUS, some can also occur as long-term sequelae of unopposed complement activation. Extra-renal symptoms are observed in approximately 20% of patients with aHUS, with the incidence of specific organ system complications ranging from a few case reports to 50% of described patients. Careful monitoring for extra-renal involvement is critical in patients with aHUS, as prompt evaluation and management may decrease the risk of high morbidity and mortality associated with aHUS.
Author(s): Cassandra Formeck [sup.1] , Agnieszka Swiatecka-Urban [sup.1] Author Affiliations: (Aff1) 0000 0004 1936 9000, grid.21925.3d, Department of Nephrology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh School of [...]