{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T14:30:34Z","timestamp":1782311434564,"version":"3.54.5"},"reference-count":24,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T00:00:00Z","timestamp":1722470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Aliment Pharmacol Ther"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background and Aims<\/jats:title><jats:p>Liver involvement is an increasingly recognised complication of common variable immunodeficiency (CVID). Nodular regenerative hyperplasia (NRH), a subgroup of porto\u2010sinusoidal vascular disorder, and manifestations of portal hypertension (PH) unrelated to cirrhosis are the most common findings. Nonetheless, the evolution of liver disease over time remains unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective review of patients followed at the National Institutes of Health with CVID\u2010related liver disease and liver biopsy from 1990 to 2020. Clinical, imaging and histological follow\u2010up were recorded as part of clinical research protocols.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty patients were included, with a median age of 37.5\u2009years at initial biopsy, 73% presenting with clear evidence of NRH, and a median fibrosis stage of 1. At biopsy, median platelet count was 100\u2009\u00d7\u200910<jats:sup>9<\/jats:sup>\/L, spleen size 19.5\u2009cm, hepatic venous pressure gradient 9.5\u2009mmHg and 37.5% of patients had signs of PH. Cumulative incidence of PH was 65% at 5\u2009years. In a subgroup of 16 patients, a follow\u2010up liver biopsy, performed at a median time of 3\u2009years after the index biopsy, revealed an increase in fibrosis by \u22652 stages in 31% of cases and an increase to an overall stage of 2.2 (<jats:italic>p<\/jats:italic>\u2009=\u20090.001). No clinical or histological factors were associated with progression of fibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In this CVID cohort, NRH is the most common initial histological finding; however, unexpectedly fibrosis progresses over time in a subgroup of patients. A better understanding of the underlying causal process of liver disease CVID might lead to improved outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apt.18180","type":"journal-article","created":{"date-parts":[[2024,8,2]],"date-time":"2024-08-02T04:49:57Z","timestamp":1722574197000},"page":"888-896","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Development of hepatic fibrosis in common variable immunodeficiency\u2010related porto\u2010sinusoidal vascular disorder"],"prefix":"10.1111","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7608-0914","authenticated-orcid":false,"given":"Julian","family":"Hercun","sequence":"first","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bilal","family":"Asif","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3855-5023","authenticated-orcid":false,"given":"Anusha","family":"Vittal","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdel","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harish Kumar","family":"Gopalakrishna\u00a0Pillai","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenna R. E.","family":"Bergerson","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Holland","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gulbu","family":"Uzel","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warren","family":"Strober","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan J.","family":"Fuss","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Immunology and Microbiology National Institute of Allergy and Infectious Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4755-5607","authenticated-orcid":false,"given":"Christopher","family":"Koh","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David E.","family":"Kleiner","sequence":"additional","affiliation":[{"name":"Laboratory of Pathology National Cancer Institute, National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theo","family":"Heller","sequence":"additional","affiliation":[{"name":"Translational Hepatology Section, Liver Diseases Branch National Institute of Diabetes and Digestive and Kidney Diseases  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.00149"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-013-9873-6"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2008.03711.x"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4725"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001452"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2007.08.011"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOG.0000000000000433"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21258"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(19)30047-0"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2021.12.022"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31775"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.05.033"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4799"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2021.05.028"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.32539"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.24979"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.25343"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(92)90771-P"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-377945"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2020.11.039"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.01.015"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2017.06.036"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.10.025"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2021.02.056"}],"container-title":["Alimentary Pharmacology &amp; 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