Common variable immunodeficiency disorder-related liver disease is common and results in portal hypertension and an increased risk of death.

ABSTRACT:

Background

Common variable immunodeficiency disorder (CVID) manifests with recurrent infections and inflammatory complications, including liver disease. We report the clinical features, natural history, and outcomes of patients with CVID-related liver disease (CVID-rLD) from a tertiary immunology and hepatology center.

Methods

Two hundred eighteen patients were identified; CVID-rLD was defined by persistently abnormal liver function tests or evidence of chronic liver disease (CLD) or portal hypertension (PHTN) by radiological or endoscopic investigation, after exclusion of other causes. Patients with CVID-rLD were investigated and managed following a joint pathway between immunology and hepatology services. Data, including clinical parameters, investigations, and outcomes, w

SUBMITTER: Halliday N 

PROVIDER: S-EPMC10727572 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature