Immunoglobulin G4-related Liver Disease Overlapping with Non-alcoholic Steatohepatitis That Was Diagnosed Simultaneously with Autoimmune Pancreatitis: A Case Report and Review of the Literature

Abstract

A 70-year-old woman was referred to our hospital due to symptoms of dry eyes, dry mouth, and epigastric pain. Computed tomography showed distal pancreatic swelling, liver edge dullness and surface irregularities. Serum anti-nuclear antibody titers, immunoglobulin G and IgG4 levels were elevated. Autoimmune pancreatitis (AIP) was diagnosed based on endoscopic findings and a histopathological examination. Her AIP improved after starting prednisolone treatment. A liver biopsy revealed interface hepatitis with lymphoplasmacyte and IgG4-positive plasma cell infiltration. In addition, non-alcoholic steatohepatitis (NASH) was diagnosed based on the presence of parenchymal steatosis, ballooning hepatocytes, and pericellular fibrosis. We experienced a unique liver disease case showing IgG4-related liver disease overlapping with NASH.

Keywords: IgG4-related disease; autoimmune hepatitis; autoimmune pancreatitis; non-alcoholic steatohepatitis; overlap.

Figure 1.

Imaging findings obtained using contrast-enhanced computed tomography (CECT), endoscopic retrograde cholangiopancreatography (ERCP), and endoscopic ultrasound (EUS). (a) CECT showed findings that were indicative of liver cirrhosis, e.g. the margins of the liver were blunted, the liver surface was irregular, and diffuse distal pancreatic swelling and a capsule-like rim were seen. (b) ERCP showed diffuse irregular narrowing of the distal main pancreatic duct and no abnormalities in the extrahepatic bile ducts. (c) EUS showed that some parts of the parenchyma of the distal pancreas were hypoechoic and lobulated, whereas other parts were swollen and exhibited a hyperechoic patchy/mesh-like appearance.

Figure 2.

Histopathological findings of the pancreas (a, b) and liver biopsies (c-h). (a, b) Marked lymphoplasmacytic cell infiltration and marked immunoglobulin G4 (IgG4) -positive plasma cell infiltration was seen in the pancreatic parenchyma. (a) (IgG staining, magnification: ×400), (b) (IgG4 staining, magnification: ×400). (c, d) Severe interface hepatitis combined with lymphoplasmacytic cell infiltration. (c) [Hematoxylin and Eosin (H&E) staining, magnification: ×100], (d) Lymphoplasmacytic cell (arrows) (H&E staining, magnification: ×400). (e) Detection of IgG4-positive plasma cells. Forty IgG4-positive plasma cells/high-power field were detected in the portal region (IgG4 staining, magnification: ×400). (f) Mild liver parenchymal inflammation and steatosis (H&E staining, magnification: ×100). (g) Ballooning hepatocytes (arrows) (H&E staining, magnification: ×400). (h) Pericellular fibrosis and bridging fibrosis (Masson’s trichrome staining, magnification: ×200)