Liver disease in primary antibody deficiencies.

Journal

Current opinion in allergy and clinical immunology

ISSN: 1473-6322

Titre abrégé: Curr Opin Allergy Clin Immunol

Pays: United States

ID NLM: 100936359

Informations de publication

Date de publication:
26 Sep 2024

Historique:

medline: 27 9 2024

pubmed: 27 9 2024

entrez: 27 9 2024

Statut: aheadofprint

Résumé

Liver disease has emerged as a major risk factor for increased mortality in patients with common variable immunodeficiency (CVID). This is mostly due to presinusoidal portal hypertension (PHTN) frequently secondary to nodular regenerative hyperplasia (NRH). Its pathogenesis is still poorly understood and treatment strategies for its various stages are often guided by trial and error. This review summarizes the most recent findings in the light of previous literature. In the last 2 years, different groups have addressed pathology, diagnostics, treatment, and liver transplantation. Histological examinations seem to support the pathogenetic sequence of T-cell mediated infiltration and damage of the sinusoidal space with secondary development of NRH, pericellular fibrosis, and the manifestation of PHTN. While markers of the early phase - beyond slight elevation of cholestatic enzymes - are still missing, elevated liver stiffness and splenomegaly above 16 cm longitudinal diameter have been suggested as warning signs for PHTN in CVID patients. Data on immunosuppressive treatment of this manifestation is still very heterogeneous, but a recent report on liver transplantation was encouraging for end stage liver disease. Liver disease deserves higher attention in the management of CVID. More studies are needed to understand its pathogenesis and optimal treatment.

Identifiants

DOI: 10.1097/ACI.0000000000001035 PMID: 39329167

pubmed: 39329167

doi: 10.1097/ACI.0000000000001035

pii: 00130832-990000000-00157

doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Informations de copyright

Références

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