PMC5399201_01
ERC showed diffuse stricture and dilatation of the intrahepatic bile duct.
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Serological testing excluded viral hepatitis and autoimmune or metabolic liver diseases, but endoscopic retrograde cholangiography (ERC) showed diffuse stricturing and dilatation of the intrahepatic bile duct (Fig. 1).
The histological findings in a liver biopsy specimen taken in October 2011. (A) Hematoxylin and Eosin staining showed interface hepatitis with marked portal lymphocytic infiltration (original magnification x400). (B) Masson trichrome staining showed slight portal fibrosis (original magnification x100). There were no findings of periductal fibrosis and cholangiopathy.
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Liver biopsy specimens revealed marked inflammation and slight fibrosis in the portal tract, as well as piecemeal necrosis; damage to the biliary epithelial cells and IgG4-positive cells were absent (Fig. 2).
The histological findings in a liver biopsy specimen taken in February 2012. (A) Hematoxylin and Eosin staining showed massive infiltration of lymphocytes and plasma cells (original magnification x400). (B) Masson trichrome staining showed fibrotic expansion in the portal tract (original magnification x100). These histopathological findings were much worse than the previous histopathological findings.
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In addition, prominent interface hepatitis and intralobular focal necrosis were noted (Fig. 3).
Changes in the levels of serum markers during the clinical course. The levels of ALT, T.Bil, and IgG decreased after the administration of PSL. ALT: alanine aminotransferase, T.Bil: total bilirubin, IgG: immunoglobulin G, PSL: prednisone
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Six weeks after starting treatment, he was discharged with amelioration of biochemical parameters, and he has been followed up with 5 mg of prednisone with normal liver biochemistry by his general practitioner (Fig. 4).
PMC5399201_02
CECT showed dilatation of the intrahepatic bile duct (arrow) and bile duct stricture from the hepatic hilar lesion to the extrahepatic bile duct with wall thickness (arrow head).
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Contrast-enhanced computed tomography (CECT) revealed dilatation of the intrahepatic bile duct and bile duct stricture from the hilar hepatic lesion to the extrahepatic bile duct with wall thickening (Fig. 5).
ERC showed dilatation of the distal intrahepatic bile duct and diffuse multiple stricturing of the hilar and intra- and extrahepatic bile ducts.
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ERC showed diffuse multiple stricturing of the hilar and intra- and extrahepatic bile ducts accompanied by dilatation of the distal bile ducts (Fig. 6).
IDUS showed circular-asymmetric wall thickness, heterogeneous internal echo, and an unclear outer margin.
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Intraductal ultrasound (IDUS) revealed circular-asymmetric wall thickness, heterogeneous internal echo, and an unclear outer margin (Fig. 7).
The histological findings in a liver biopsy specimen taken in March 2015. Hematoxylin and Eosin staining showed mild interface hepatitis with portal inflammation and focal intralobular necrosis (original magnification A: x200, B: x400). (C) Masson trichrome staining showed no findings of biliary fibrosis (original magnification x100).
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Biliary fibrosis, neoplastic lesion, and IgG4-positive cells were not observed (Fig. 8).
The histological findings in a liver biopsy specimen taken in June 2015. Hematoxylin and Eosin staining showed prominent necroinflammatory features with severe interface hepatitis and multiple interlobular focal necroses, as well as periportal fibrosis. (C) Masson trichrome staining showed no characteristic periductal onion-skin fibrosis (original magnification x100).
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A characteristic periductal 'onion-skin' fibrosis was not observed (Fig. 9).
Follow-up MRCP did not show any changes in the biliary lesions 6 weeks after the start of steroid treatment.
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Magnetic resonance cholangiopancreatography (MRCP) showed no marked changes in the biliary lesions in 6 weeks (Fig. 10).
Changes in the levels of serum markers during the clinical course. The levels of ALT, T.Bil, and IgG decreased after the administration of mPSL and PSL. ALT: alanine aminotransferase, T.Bil: total bilirubin, IgG: immunoglobulin G, mPSL: methylprednisolone, PSL: prednisone
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The prednisone was tapered to 15 mg 8 months later at the outpatient clinic with very slight elevation in his liver biochemistry findings (Fig. 11).