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Previous Volume 344:732-738 March 8, 2001 Number 10 Next

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ABSTRACT

Background Sclerosing pancreatitis is a unique form of pancreatitis that is characterized by irregular narrowing of the main pancreatic duct, lymphoplasmacytic inflammation of the pancreas, and hypergammaglobulinemia and that responds to glucocorticoid treatment. Preliminary studies suggested that serum IgG4 concentrations are elevated in this disease but not in other diseases of the pancreas or biliary tract.

Methods We measured serum IgG4 concentrations using single radial immunodiffusion and an enzyme-linked immunosorbent assay in 20 patients with sclerosing pancreatitis, 20 age- and sex-matched normal subjects, and 154 patients with pancreatic cancer, ordinary chronic pancreatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or Sjögren's syndrome. Serum concentrations of immune complexes and the IgG4 subclass of immune complexes were determined by means of an enzyme-linked immunosorbent assay with monoclonal rheumatoid factor.

Results The median serum IgG4 concentration in the patients with sclerosing pancreatitis was 663 mg per deciliter (5th and 95th percentiles, 136 and 1150), as compared with 51 mg per deciliter (5th and 95th percentiles, 15 and 128) in normal subjects (P<0.001). The serum IgG4 concentrations in the other groups of patients were similar to those in the normal subjects. In patients with sclerosing pancreatitis, serum concentrations of immune complexes and the IgG4 subclass of immune complexes were significantly higher before glucocorticoid therapy than after four weeks of such therapy. Glucocorticoid therapy induced clinical remissions and significantly decreased serum concentrations of IgG4, immune complexes, and the IgG4 subclass of immune complexes.

Conclusions Patients with sclerosing pancreatitis have high serum IgG4 concentrations, providing a useful means of distinguishing this disorder from other diseases of the pancreas or biliary tract.

Source Information

From the Second Department of Internal Medicine (H.H., S.K., A.H., H.U., N.F., T.A., K.K.) and the Departments of Laboratory Medicine (M.F.), Obstetrics and Gynecology (T.N.), Organ Generation (T.N.), and Anatomy (K.N.), Shinshu University School of Medicine, Matsumoto, Japan; and the Department of Anatomy, Fujita Health University School of Medicine, Toyoake, Japan (N.U.).

Address reprint requests to Dr. Kawa at the Second Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan, or at skawapc{at}hsp.md.shinshu-u.ac.jp.

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• (2001). Sclerosing Pancreatitis: A Unique Disease Associated with High IgG4 Concentrations. JWatch Gastroenterology 2001: 6-6 [Full Text]