Autoimmune hepatitis-primary biliary cholangitis overlap syndrome complicated by various autoimmune diseases: A case report

Abstract

Background: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two common clinical autoimmune liver diseases, and some patients have both diseases; this feature is called AIH-PBC overlap syndrome. Autoimmune thyroid disease (AITD) is the most frequently overlapping extrahepatic autoimmune disease. Immunoglobulin (IgG) 4-related disease is an autoimmune disease recognized in recent years, characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.

Case summary: A 68-year-old female patient was admitted with a history of right upper quadrant pain, anorexia, and jaundice on physical examination. Laboratory examination revealed elevated liver enzymes, multiple positive autoantibodies associated with liver and thyroid disease, and imaging and biopsy suggestive of pancreatitis, hepatitis, and PBC. A diagnosis was made of a rare and complex overlap syndrome of AIH, PBC, AITD, and IgG4-related disease. Laboratory features improved on treatment with ursodeoxycholic acid, methylprednisolone, and azathioprine.

Conclusion: This case highlights the importance of screening patients with autoimmune diseases for related conditions.

Keywords: Autoimmune hepatitis; Autoimmune thyroid disease; Case report; Overlap syndrome; Primary biliary cholangitis; Primary sclerosing cholangitis.

Figure 1

Biochemical indicators of patients. A: Dynamic changes in ALT; B: Dynamic changes in AST; C: Dynamic changes in TBA and GGT; D: Dynamic changes in TBIL and DBIL; E: Dynamic changes in serum IgG; F: Dynamic changes in serum IgA and IgM. ALT: Alanine aminotransferase; UDCA: Ursodeoxycholic acid; AST: Aspartate aminotransferase; TBA: Total bile acid; GGT: Glutamyl transpeptidase; TBIL: Total bilirubin; DBIL: Direct bilirubin; IgG: Immunoglobulin G; IgA: Immunoglobulin A; IgM: Immunoglobulin M.

Figure 2

Patient magnetic resonance imaging findings. A: Multiple enlarged lymph nodes in the hepatic hilum at admission; B: The hilar lymphadenopathy resolved after 8 wk of treatment; C: Pancreatic duct dilatation at admission; D: Pancreatic duct dilatation persists after 8 wk of treatment; E: Enlargement of the head of the pancreas at admission; F: Pancreatic head enlargement has been significantly reduced after 8 wk of treatment.

Figure 3

Patient pathological manifestations. A: Plentiful lymphocytes and plasma cells can be seen in a lymph node of the porta hepatis; B: Liver tissue HE staining; scattered punctate necrosis of the liver and infiltration of a large number of inflammatory cells can be seen (HE × 100); C: Liver tissue Masson staining; visible portal fibrosis with fibrous septa formation can be seen (IHC × 100); D: Inflammatory cell infiltration in the portal area of the liver tissue; liver tissue immunoglobulin (IgG) 4-negative (IHC × 200); E: Inflammatory cell infiltration in the portal area of the liver tissue; liver tissue IgG-positive (IHC × 200); F:Pancreatic HE staining; small amount of inflammatory cell infiltration can be seen in the glands of the pancreas (HE × 100); G: Pancreatic IgG4-negative (IHC × 200).

Figure 4

Endoscopic ultrasound findings of patients. A: Endoscopic ultrasonography of hepatic lymph node; B: Uncinate process of the pancreas; C: Pancreatic lymph node; D: Pancreatic lymph node.