Autoimmune pancreatitis.
Although the pathogenesis of autoimmune pancreatitis is unclear, recent evidence of clinical aspects are presented: (i) mild abdominal symptoms, usually without acute attacks of pancreatitis; (ii) occasional existence of obstructive jaundice; (iii) increased levels of serum gammaglobulin, IgG or IgG4; (iv) presence of autoantibodies; (v) diffuse enlargement of the pancreas; (vi) irregularly narrowing of the pancreatic duct (sclerosing pancreatitis) with often intra-pancreatic biliary stenosis or coexistence of biliary lesions (sclerosing cholangitis similar to primary sclerosing cholangitis: PSC) on endoscopic retrograde cholangiopancreatographic (ERCP) images; (vii) fibrotic changes with lymphocyte and IgG4-positive plasmacyte infiltration, and obliterative thrombo-phlebitis; (viii) occasional association with other systemic lesions such as sialoadenitis, retroperitoneal fibrosis, interstitial renal tubular disorders, and (ix) effective steroid therapy. In addition to pancreatic and extra-pancreatic lesions, diabetes mellitus is occasionally responsive to steroid therapy. Further studies are needed to clarify the pathogenesis.
Okazaki
Kazuichi
K
Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan. okazaki@taku.kmu.ac.jp
Uchida
Kazushige
K
Matsushita
Mitsunobu
M
Takaoka
Makoto
M
D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases; D015415:Biomarkers / Q000097:blood; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D010195:Pancreatitis / Q000097:blood / Q000175:diagnosis / Q000276:immunology*; D005719:gamma-Globulins / Q000378:metabolism
D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G; D005719:gamma-Globulins