title: IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp
authors.forename: T
authors.initials: T
authors.lastname: Kamisawa
authors.forename: H
authors.initials: H
authors.lastname: Nakajima
authors.forename: N
authors.initials: N
authors.lastname: Egawa
authors.forename: N
authors.initials: N
authors.lastname: Funata
authors.forename: K
authors.initials: K
authors.lastname: Tsuruta
authors.forename: A
authors.initials: A
authors.lastname: Okamoto
mesh_terms: 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
chemical_list: D007074:Immunoglobulin G