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


CONCLUSIONS
We propose a new clinicopathological entity of IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy.
Kamisawa
T
T
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp
Nakajima
H
H
Egawa
N
N
Funata
N
N
Tsuruta
K
K
Okamoto
A
A
D000368:Aged; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology; D002761:Cholangitis / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000276:immunology*; D007150:Immunohistochemistry; D008206:Lymphatic Diseases / Q000276:immunology* / Q000473:pathology; D008297:Male; D011446:Prospective Studies; D012185:Retroperitoneal Fibrosis / Q000276:immunology* / Q000473:pathology; D012793:Sialadenitis / Q000276:immunology* / Q000473:pathology; D013344:Subacute Sclerosing Panencephalitis / Q000276:immunology* / Q000473:pathology; D013577:Syndrome
D007074:Immunoglobulin G