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Review
. 2019 Aug;34(8):1337-1348.
doi: 10.1007/s00467-018-4039-7. Epub 2018 Aug 14.

Extra-renal manifestations of atypical hemolytic uremic syndrome

Cassandra Formeck  1 , Agnieszka Swiatecka-Urban  2
Affiliations
Review

Extra-renal manifestations of atypical hemolytic uremic syndrome

Cassandra Formeck et al. Pediatr Nephrol. 2019 Aug.

Abstract

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.

Keywords: Atypical hemolytic-uremic syndrome (aHUS); Cardiovascular complications; Extra-renal manifestations; Gastrointestinal complications; Neurologic complications; Thrombotic microangiopathy (TMA).

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Conflict of interest statement

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Conflict of interest

The authors declare no conflict of interest

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