Primary sclerosing cholangitis associated with elevated immunoglobulin-g4: a preliminary study

Area of Science:

• Gastroenterology
• Immunology
• Hepatology

Background:

• Immunoglobulin IgG4-associated cholangitis (IAC) is a systemic disease characterized by T lymphocyte and IgG4-positive plasma cell infiltration.
• Previous studies reported IAC prevalence in primary sclerosing cholangitis (PSC) patients between 7% and 11.6%.

Purpose of the Study:

• To evaluate the frequency of elevated serum IgG4 levels in PSC patients.
• To compare clinical, laboratory, and imaging findings between PSC patients with high IgG4 levels and those with normal levels.

Main Methods:

• A prospective analytical cross-sectional study was conducted.
• 34 newly diagnosed PSC patients were assessed for serum IgG4 levels.
• Clinical presentation, laboratory values, imaging, and comorbidities were analyzed.

Main Results:

• 9 out of 34 PSC patients (26.5%) exhibited elevated serum IgG4 levels.
• No significant differences were observed in age, smoking status, IBD, ascites, esophageal varices, Child score, or imaging findings between PSC patients with high or normal IgG4 levels.
• The majority of patients were male (67.6%) and non-smokers (76.5%).

Conclusions:

• A higher frequency of elevated serum IgG4 was observed in PSC patients than previously reported.
• IAC should be considered in PSC patients with unexplained biliary strictures and elevated serum IgG4.
• Future studies should consider testing PSC patients for IgG4 and evaluating corticosteroid treatment for those with high levels.