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Atypical Haemolytic Uraemic Syndrome

Lookup NU author(s): Dr Patrick WalshORCiD, Professor David KavanaghORCiD

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Abstract

The Haemolytic Uremic Syndromes (HUS) are a heterogeneous group of conditions with differing underlying pathophysiology. These all result in the pathological features of thrombotic microangiopathy (TMA) which causes endothelial damage and organ injury. TMAs manifest with a microangiopathic haemolytic anaemia, thrombocytopenia and commonly acute kidney injury. The past two decades have revealed many of the immunological, metabolic, ribosomal, cell signalling and clotting pathways responsible for disease. The understanding of the role of membrane attack complex in some TMAs led to the introduction of pharmacological inhibition of Complement C5, which greatly improved prognosis. This review summaries our current understanding of the pathogenesis of complement dependent and non-complement dependent TMAs.

Publication metadata

Author(s): Walsh PR, Kavanagh D

Publication type: Article

Publication status: Published

Journal: Journal of Allergy and Clinical Immunology

Year: 2025

Volume: 156

Issue: 2

Pages: 224-236

Print publication date: 01/08/2025

Online publication date: 26/05/2025

Acceptance date: 02/05/2025

Date deposited: 16/06/2025

ISSN (print): 0091-6749

ISSN (electronic): 1097-6825

Publisher: Elsevier

URL: https://doi.org/10.1016/j.jaci.2025.05.010

DOI: 10.1016/j.jaci.2025.05.010

PubMed id: 40436118

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