• Ir J Med Sci · Aug 2023

    Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis.

    • John P ONeill, Cathal S Clifford, Niamh P Nolan, and McCormickP AidenPA0000-0002-5994-6023Liver Unit, St Vincent's University Hospital, Dublin, D04 T6F4, Ireland. a.mccormick@ucd.ie.University College Dublin, Dublin, D04 V1W8, Ireland. a.mccormick@ucd.ie..
    • Department of Histopathology, St Vincent's University Hospital, Dublin, D04 T6F4, Ireland.
    • Ir J Med Sci. 2023 Aug 1; 192 (4): 165716601657-1660.

    BackgroundCystic fibrosis (CF)-associated liver disease commonly manifests as portal hypertension and its complications. We investigated the proposal that the pathophysiology is of non-cirrhotic rather than cirrhotic portal hypertension. This distinction may have important implications for treatment.MethodsWe compared liver transplant explants from cystic fibrosis patients with explants from patients with classical cholestatic diseases, primary biliary cholangitis and primary sclerosing cholangitis. Presence of cirrhosis, fibrosis, nodular regenerative hyperplasia, biliary and portal venous pathology were recorded. Quantitation of portal venules in representative section was performed.ResultsNine patients with cystic fibrosis liver disease, 7 primary biliary cholangitis (PBC) and 7 primary sclerosing cholangitis (PSC) were evaluated. Cirrhosis was present in 0/9 of CF patients and 11/14 of the PBC and PSC controls (p < 0.01). Nodular regenerative hyperplasia was present in 8/9 of the CF patients but none of the controls (p < 0.01). Portal venule numbers per 15 mm2 section were significantly lower in the CF patients 52 (20-72) compared to the primary biliary cholangitis 78 (47-110) and primary sclerosing cholangitis patients, 79 (41-134) (p < 0.05). Portal sclerotic nodules were found in all the CF patients but in only one of the controls (9/9 vs 1/14 p < 0.01).ConclusionsThis study demonstrates that non-cirrhotic portal hypertension or obliterative portal venopathy is the predominant hepatic pathophysiology in adult CF patients requiring liver transplantation. It suggests that treatments directed at the hepatic portal venous system may be more effective than current treatment directed at the biliary system in cystic fibrosis.© 2022. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.

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