IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy.

Abstract

BACKGROUND AND AIMS:

Autoimmune pancreatitis is usually associated with elevated serum IgG4 concentrations, and sometimes with sclerosing cholangitis and Sjögren's syndrome. This study aimed to elucidate the proposed entity of IgG4-related sclerosing disease.

METHODS:

Subjects were patients with autoimmune pancreatitis (n = 26), sclerosing sialadenitis (n = 5), chronic alcoholic pancreatitis (n = 20), sialolithiasis (n = 34), Sjögren's syndrome (n = 50), and primary sclerosing cholangitis (n = 3). Sections of various organs and tissues of these patients were examined immunohistochemically using antibodies to CD4-T, CD8-T, and CD20-B cell subsets and IgG4, and serum IgG4 concentrations were measured.

RESULTS:

Patients with autoimmune pancreatitis were associated with sclerosing cholangitis (n = 23), sclerosing sialadenitis (n = 2), retroperitoneal fibrosis (n = 2), and abdominal (n = 5) and cervical (n = 4) lymphadenopathy. They demonstrated infiltrations of more abundant IgG4-positive plasma cells in the pancreas, peripancreatic retroperitoneal tissues, extrahepatic bile duct, gallbladder, stomach, minor salivary gland, and abdominal lymph nodes compared with those of other diseases (p < 0.01). Such infiltrations were also observed in the minor salivary gland and submandibular gland of patients with sclerosing sialadenitis (p < 0.01). Serum IgG4 concentrations were significantly elevated in patients with autoimmune pancreatitis and sclerosing sialadenitis (p < 0.01).

CONCLUSION:

We propose a new clinicopathological entity of IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy.

• Authors: T Kamisawa, H Nakajima, N Egawa, N Funata, K Tsuruta, A Okamoto
• Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] (Pancreatology) Vol. 6 Issue 1-2 Pg. 132-7 ( 2006) ISSN: 1424-3903 [Print] Switzerland
• PMID: 16327291 (Publication Type: Journal Article)
• Copyright: 2006 S. Karger AG, Basel and IAP

Chemical References

• Immunoglobulin G

Topics

• Aged
• Autoimmune Diseases (immunology, pathology)
• Cholangitis (immunology, pathology)
• Female
• Humans
• Immunoglobulin G (analysis, immunology)
• Immunohistochemistry
• Lymphatic Diseases (immunology, pathology)
• Male
• Prospective Studies
• Retroperitoneal Fibrosis (immunology, pathology)
• Sialadenitis (immunology, pathology)
• Subacute Sclerosing Panencephalitis (immunology, pathology)
• Syndrome