PMC7388380_01
(A) Moderate chronic inflammation of the portal tract associated with epithelioid cells adjacent to the bile ducts and ductal proliferation. (B) Severe inflammation of the portal tract, with diffuse interface hepatitis and plasma cells (arrows). Ductal inflammation and ductal damage. (C) Severe chronic portal and interface hepatitis, with presence of plasma cells near the limiting plate (arrows). Ductal epithelium shows mild atypia, associated with inflammation. Severe architectural distortion compatible with cirrhosis. (D) Multifocal stenosis and dilatation of the intrahepatic bile ducts involving the second and third liver segments (E) Persistence of lympho-histiocytic infiltrate in the portal area, associated with interface hepatitis. A few plasma-cells (arrows) are present near the limiting plate.
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Owing to these laboratory results, the patient underwent a liver biopsy, which showed florid bile duct lesions (Ludwig stage II PBC) (Fig. 1A). Five years later, a follow-up liver biopsy showed stage III PBC and appearance of interface hepatitis (Fig. 1B). Twelve months after starting treatment, a liver biopsy showed worsening of portal and interface hepatitis, markedly increased fibrosis, and bile duct proliferation (Fig. 1C). MRCP, performed to exclude bile duct obstruction in view of poor response to treatment, showed intrahepatic bile duct changes characteristic of sclerosing cholangitis (Fig. 1D). After five years of MMF/UDCA therapy, blood tests remain normal (including normalization of GGT and IgM levels), apart from AMA and transitory re-appearance of rim-like ANA positivity, without signs of portal hypertension, though a liver biopsy performed after one year of MMF/UDCA treatment, showed persistent interface hepatitis and advanced fibrosis (Fig. 1E).