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Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis
Flavia D Mendes1, Roberta Jorgensen, Jill Keach
1Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
The American Journal of Gastroenterology
|August 2, 2006
Summary
A small percentage of primary sclerosing cholangitis (PSC) patients have elevated immunoglobulin G4 (IgG4) levels, indicating a more severe disease course and shorter time to liver transplantation. Further research into IgG4 testing and corticosteroid treatment for PSC is warranted.
Area of Science:
- Hepatology
- Immunology
Background:
- Biliary strictures in autoimmune pancreatitis are linked to elevated serum IgG4 and corticosteroid responsiveness.
- Primary sclerosing cholangitis (PSC) shares some features with autoimmune pancreatitis.
Purpose of the Study:
- To determine the frequency of elevated serum IgG4 in PSC patients.
- To compare clinical characteristics of PSC patients with normal versus elevated IgG4 levels.
Main Methods:
- Serum IgG4 levels were measured in 127 PSC patients and 87 primary biliary cirrhosis (PBC) controls.
- Demographic, clinical, and laboratory data were compared between PSC groups with normal and elevated IgG4 (>140 mg/dL).
Main Results:
- Elevated IgG4 was observed in 9% of PSC patients versus 1.1% of PBC patients (p=0.017).
- PSC patients with elevated IgG4 exhibited higher bilirubin, alkaline phosphatase, and PSC Mayo risk scores, with less frequent IBD.
- The median time to liver transplantation was significantly shorter for PSC patients with elevated IgG4 (1.7 years vs. 6.5 years, p=0.0009).
Conclusions:
- A subset of PSC patients shows elevated serum IgG4, correlating with increased liver disease severity and a more rapid progression towards transplantation.
- These findings suggest that elevated IgG4 in PSC may indicate a distinct disease phenotype, potentially responsive to corticosteroids, similar to IgG4-related sclerosing cholangitis.
- Clinical trials investigating IgG4 testing and corticosteroid treatment in PSC patients with elevated IgG4 are recommended.
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