A new clinicopathological entity of IgG4-related autoimmune disease.
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.
Kamisawa
Terumi
T
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan.
Funata
Nobuaki
N
Hayashi
Yukiko
Y
Eishi
Yoshinobu
Y
Koike
Morio
M
Tsuruta
Kouji
K
Okamoto
Atsutake
A
Egawa
Naoto
N
Nakajima
Hitoshi
H
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
D000944:Antigens, Differentiation, B-Lymphocyte; D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; D007074:Immunoglobulin G