title: A new clinicopathological entity of IgG4-related autoimmune disease.
abstract: BACKGROUND
  Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.
  
  
  METHODS
  To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies.
  
  
  RESULTS
  In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls.
  
  
  CONCLUSIONS
  These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.
authors: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan.
authors: Terumi
authors: T
authors: Kamisawa
authors: Nobuaki
authors: N
authors: Funata
authors: Yukiko
authors: Y
authors: Hayashi
authors: Yoshinobu
authors: Y
authors: Eishi
authors: Morio
authors: M
authors: Koike
authors: Kouji
authors: K
authors: Tsuruta
authors: Atsutake
authors: A
authors: Okamoto
authors: Naoto
authors: N
authors: Egawa
authors: Hitoshi
authors: H
authors: Nakajima
mesh_terms: D000368:Aged; D000944:Antigens, Differentiation, B-Lymphocyte / Q000097:blood / Q000276:immunology; D000945:Antigens, Differentiation, T-Lymphocyte / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology* / Q000503:physiopathology*; D001652:Bile Ducts / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D015415:Biomarkers / Q000097:blood; D002908:Chronic Disease; D003106:Colon / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D005260:Female; D005753:Gastric Mucosa / Q000378:metabolism; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D007150:Immunohistochemistry; D008099:Liver / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008198:Lymph Nodes / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000097:blood / Q000276:immunology / Q000503:physiopathology; D010950:Plasma Cells / Q000276:immunology / Q000378:metabolism; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000276:immunology / Q000503:physiopathology; D012469:Salivary Glands / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D012720:Severity of Illness Index; D013223:Statistics as Topic; D013270:Stomach / Q000276:immunology / Q000473:pathology
chemical_list: D000944:Antigens, Differentiation, B-Lymphocyte; D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; D007074:Immunoglobulin G