case_images: PMC9894089_01
case_images: Magnetic resonance cholangiography suggesting definite changes of sclerosing cholangitis
case_images: CEJI-47-47053-g001.jpg
case_images: PMC9894089_01_CEJI-47-47053-g001.jpg
case_images: F1
case_images: The patient was further investigated with magnetic resonance cholangiopancreatography (MRCP), which demonstrated heterogeneous liver enhancement with an increased T2 signal predominantly in the left hepatic lobe, multifocal stricturing and dilation of intrahepatic and extrahepatic bile ducts (Fig. 1).
case_images: Liver histology and immunohistochemistry of tissue material obtained from liver biopsy in a child with ASC. A) Section showing marked chronic portal and periportal intense inflammation in most portal tracts, composed of lymphocytes and plasma cells, with marked interface hepatitis (Mayer's hematoxylin and eosin - H&E staining, original magnification 100x). B) The view of intense mononuclear infiltrates and expansion of collagen fibers around numerous, proliferating small bile ducts present within the liver scar formation, giving the morphological pattern of small-duct (autoimmune) sclerosing cholangitis (Masson's trichrome staining, original magnification 200x). Immunohistochemical staining for CK7 well demonstrates the proliferation of numerous bile ductules within the scar formation; abundant mononuclear inflammatory cell infiltrations are present around the neoforming small ducts (IHC, original magnification: 100x (C); 200x (D))
case_images: CEJI-47-47053-g002.jpg
case_images: PMC9894089_01_CEJI-47-47053-g002.jpg
case_images: F2
case_images: Characteristic features for AIH were found, i.e. interface and lobular hepatitis, moderate/severe in nature, with intense portal and periportal lymphoplasmacytic infiltrates and severe necro-inflammatory reaction (Fig. 2A). The process of intense fibrogenesis was accompanied by an abundant amount of mononuclear inflammatory cells in the collagen fibrous tissue (Fig. 2B). Immunohistochemical (IHC) staining of the portal tract for CK7 and CK19 revealed a proliferation of the biliary tree, which morphologically corresponds to small-duct sclerosing cholangitis (Fig. 2C, D).