A new clinicopathological entity of IgG4-related autoimmune disease

• Published in: Journal of gastroenterology Vol. 38; no. 10; pp. 982 - 984
• Main Authors: Kamisawa, Terumi, Funata, Nobuaki, Hayashi, Yukiko, Eishi, Yoshinobu, Koike, Morio, Tsuruta, Kouji, Okamoto, Atsutake, Egawa, Naoto, Nakajima, Hitoshi
• Format: Journal Article
• Language: English
• Published: Japan Springer Nature B.V 01.10.2003
• Subjects: Aged; Antigens, Differentiation, B-Lymphocyte - blood; Antigens, Differentiation, B-Lymphocyte - immunology; Antigens, Differentiation, T-Lymphocyte - blood; Antigens, Differentiation, T-Lymphocyte - immunology; Autoimmune Diseases - blood; Autoimmune Diseases - immunology; Autoimmune Diseases - physiopathology; Bile Ducts - immunology; Bile Ducts - metabolism; Bile Ducts - pathology; Biomarkers - blood; Chronic Disease; Colon - immunology; Colon - metabolism; Colon - pathology; Female; Humans; Immunoglobulin G - blood; Immunoglobulin G - immunology; Immunohistochemistry; Liver - immunology; Liver - metabolism; Liver - pathology; Lymph Nodes - immunology; Lymph Nodes - metabolism; Lymph Nodes - pathology; Male; Middle Aged; Pancreatitis - blood; Pancreatitis - immunology; Pancreatitis - physiopathology; Plasma Cells - immunology; Plasma Cells - metabolism; Retroperitoneal Fibrosis - blood; Retroperitoneal Fibrosis - immunology; Retroperitoneal Fibrosis - physiopathology; Salivary Glands - immunology; Salivary Glands - metabolism; Salivary Glands - pathology; Severity of Illness Index; Statistics as Topic; Stomach - immunology; Stomach - metabolism; Stomach - pathology
• ISSN: 0944-1174; 1435-5922
• DOI: 10.1007/s00535-003-1175-y

Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. 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. 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. 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.