IgG4-related autoimmune hepatitis: A case report.

Area of Science:

• Hepatology
• Immunology
• Internal Medicine

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition affecting multiple organs.
• IgG4-related autoimmune hepatitis (IgG4-AIH) is a rare manifestation of IgG4-RD, characterized by elevated serum IgG4 levels and specific histopathological findings.
• Accurate diagnosis is crucial for appropriate management and to differentiate from other causes of hepatitis.

Observation:

• An elderly male presented with unexplained hepatic insufficiency.
• Extensive investigations excluded common causes of liver disease, including viral, alcoholic, and drug-induced hepatitis.
• Elevated serum IgG4, abnormal humoral immunity indices, a distinct antibody spectrum, and characteristic liver biopsy findings supported the diagnosis.

Findings:

• The patient was diagnosed with IgG4-related autoimmune hepatitis (IgG4-AIH).
• Treatment with prednisone and ursodeoxycholic acid was initiated.
• The patient demonstrated a significant improvement in liver function following treatment.

Implications:

• This case highlights the importance of considering IgG4-AIH in elderly patients with unexplained liver dysfunction.
• Early diagnosis and immunosuppressive therapy can lead to favorable outcomes in IgG4-AIH.
• Further research is needed to understand the pathogenesis and optimize treatment strategies for IgG4-AIH.