ArticleFrontiers in immunology2022
Liver disease accompanied by enteropathy in common variable immunodeficiency: Common pathophysiological mechanisms.
Article in Frontiers in immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 22 citations in OpenAlex.
- Clinical Spectrum and Therapeutic Options in Monogenic CTLA-4-, LRBA-, or SOCS1-Related Primary Immune Regulatory Disorders.The journal of allergy and clinical immunology. In practice · 2026Review
- Porto-sinusoidal Vascular Disease is Associated with Gastrointestinal Disorders in Common Variable Immunodefiency.Journal of clinical immunology · 2026Article
- Liver Disease in Common Variable Immunodeficiency: Current Evidence and Knowledge Gaps.International journal of molecular sciences · 2026Review
- Non-invasive assessment of hepatic involvement in common variable immunodeficiency and agammaglobulinemia using enhanced liver fibrosis score and shearwave elastography.Frontiers in immunology · 2026Article
- Severe Bleeding From Portal Hypertension in a Young Patient With Common Variable Immunodeficiency-Associated Nodular Regenerative Hyperplasia.ACG case reports journal · 2025Article
- Hepatic Manifestations in Common Variable Immunodeficiency Associated With Mortality and Worse Hospital Outcomes in Nationwide Analysis Using National Readmission Database.The journal of allergy and clinical immunology. In practice · 2025Article
- A Broad Spectrum of Liver Manifestations in Common Variable Immunodeficiency Syndrome-Two Case Reports and a Literature Overview.Diagnostics (Basel, Switzerland) · 2025Article
- The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency.International journal of molecular sciences · 2025Review
- Development and validation of a nomogram for predicting immune-mediated colitis in lung cancer patients treated with immune checkpoint inhibitors: a retrospective cohort study in China.Frontiers in immunology · 2025Article
- Common variable immunodeficiency disorder-related liver disease is common and results in portal hypertension and an increased risk of death.Hepatology communications · 2024Article
- Differential Diagnosis: Hepatic Complications in Inborn Errors of Immunity.Journal of clinical medicine · 2023Review
- Management of liver disease and portal hypertension in common variable immunodeficiency (CVID).JHEP reports : innovation in hepatology · 2023Review
- Is there a role for microbiome-based approach in common variable immunodeficiency?Clinical and experimental medicine · 2023Review
- Portal hypertension in common variable immunodeficiency disorders - a single center analysis on clinical and immunological parameter in 196 patients.Frontiers in immunology · 2023Article
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Common variable immunodeficiency (CVID) is one of the inborn errors of immunity that have the greatest clinical impact. Rates of morbidity and mortality are higher in patients with CVID who develop liver disease than in those who do not. The main liver disorder in CVID is nodular regenerative hyperplasia (NRH), the cause of which remains unclear and for which there is as yet no treatment. The etiology of liver disease in CVID is determined by analyzing the liver injury and the associated conditions. The objective of this study was to compare CVID patients with and without liver-spleen axis abnormalities in terms of clinical characteristics, as well as to analyze liver and duodenal biopsies from those with portal hypertension (PH), to elucidate the pathophysiology of liver injury. Patients were divided into three groups: Those with liver disease/PH, those with isolated splenomegaly, and those without liver-spleen axis abnormalities. Clinical and biochemical data were collected. Among 141 CVID patients, 46 (32.6%) had liver disease/PH; 27 (19.1%) had isolated splenomegaly; and 68 (48.2%) had no liver-spleen axis abnormalities. Among the liver disease/PH group, patients, even those with mild or no biochemical changes, had clinical manifestations of PH, mainly splenomegaly, thrombocytopenia, and esophageal varices. Duodenal celiac pattern was found to correlate with PH (p < 0.001). We identified NRH in the livers of all patients with PH (
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