ArticleJournal of clinical immunology2022
Evaluation of Laboratory and Sonographic Parameters for Detection of Portal Hypertension in Patients with Common Variable Immunodeficiency.
Article in Journal of clinical immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 14 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
- A high FIB-4 score may be an early sign of severe liver involvement in patients with primary antibody deficiencies.Turkish journal of medical sciences · 2026Article
- Non-invasive assessment of hepatic involvement in common variable immunodeficiency and agammaglobulinemia using enhanced liver fibrosis score and shearwave elastography.Frontiers in immunology · 2026Article
- 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
- Severe Bleeding From Portal Hypertension in a Young Patient With Common Variable Immunodeficiency-Associated Nodular Regenerative Hyperplasia.ACG case reports journal · 2025Article
- Common variable immunodeficiency disorder-related liver disease is common and results in portal hypertension and an increased risk of death.Hepatology communications · 2024Article
- Management of liver disease and portal hypertension in common variable immunodeficiency (CVID).JHEP reports : innovation in hepatology · 2023Review
- Portal hypertension in common variable immunodeficiency disorders - a single center analysis on clinical and immunological parameter in 196 patients.Frontiers in immunology · 2023Article
- Clinical manifestations and approach to the management of patients with common variable immunodeficiency and liver disease.Frontiers in immunology · 2023Article
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Authors and funding
16 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Timely detection of portal hypertension as a manifestation in a subgroup of patients with common variable immunodeficiency (CVID) represents a challenge since it is usually not associated with liver cirrhosis. To identify relevant markers for portal hypertension, we evaluated clinical history, laboratory parameters, and abdominal ultrasound including liver elastography and biomarkers of extracellular matrix formation. Twenty seven (6%) of 479 CVID patients presented with clinically significant portal hypertension as defined by either the presence of esophageal varices or ascites. This manifestation occurred late during the course of the disease (11.8 years after first diagnosis of CVID) and was typically part of a multiorgan disease and associated with a high mortality (11/27 patients died during follow up). The strongest association with portal hypertension was found for splenomegaly with a longitudinal diameter of > 16 cm. Similarly, most patients presented with a liver stiffness measurement (LSM) of above 6.5 kPa, and a LSM above 20 kPa was always indicative of manifest portal hypertension. Additionally, many laboratory parameters including Pro-C4 were significantly altered in patients with portal hypertension without clearly increasing the discriminatory power to detect non-cirrhotic portal hypertension in CVID. Our data suggest that a spleen size above 16 cm and an elevated liver stiffness above 6.5 kPa should prompt further evaluation of portal hypertension and its sequelae, but earlier and better liquid biomarkers of this serious secondary complication in CVID are needed.
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