[Isolated IgG4-related sclerosing cholangitis]

Area of Science:

• Gastroenterology
• Hepatology
• Immunology

Background:

• IgG4-related sclerosing cholangitis (IAC) is a rare fibrotic condition.
• Distinguishing IAC from other biliary strictures, such as cholangiocarcinoma, is crucial for appropriate management.

Purpose of the Study:

• To describe the clinical characteristics of five patients with isolated IAC.
• To review diagnostic approaches and treatment outcomes for this rare condition.

Main Methods:

• Retrospective analysis of clinical data, laboratory tests, imaging, and liver pathology in five patients with isolated IAC.
• Evaluation of treatment responses and prognosis.

Main Results:

• All patients presented with jaundice and elevated serum IgG4 levels.
• Imaging revealed bile duct stenosis in all cases; two patients had pancreatic enlargement.
• Liver biopsy confirmed fibrosis and IgG4-positive plasma cell infiltration.
• Steroid therapy led to symptom relief and no recurrence during follow-up.

Conclusions:

• Isolated IAC is a rare entity that can mimic cholangiocarcinoma.
• Surgical intervention is often ineffective and should be avoided.
• Consider IAC in patients with long-standing, fluctuating jaundice and pancreatic enlargement.
• Serum IgG4 levels and liver biopsy are key diagnostic tools.