2022 Volume 63 Issue 2 Pages 43-50
An 80-year-old man presented to our hospital complaining of epigastric pain. His levels of hepatobiliary system enzymes were elevated. Computed tomography (CT) showed no obvious common bile duct stones but mild bile duct dilatation was present. He was admitted to the hospital for treatment of suspected cholangitis. Repeated endoscopic bile duct drainage did not improve the liver damage. Although serum immunoglobulin (Ig) G was mildly elevated, viral markers and autoantibodies were negative. Liver biopsy showed nonspecific, chronic active hepatitis but did not lead to a definitive diagnosis. Additional blood tests showed high IgG4 levels and IgG4 immunostaining of liver biopsy pathology showed IgG4-positive plasma cell infiltration in the portal. Based on the above, the patient was diagnosed with IgG4-related autoimmune hepatitis, and prednisolone (PSL) was started; the liver damage promptly improved. This case demonstrates that measurement of serum IgG4 and IgG4 immunostaining of liver tissue may be useful for the diagnosis of liver disorders of unknown origin.
