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Autoimmune pancreatitis : ウィキペディア英語版
Autoimmune pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment with corticosteroids, particularly prednisone. There are two categories of AIP: Type 1 and Type 2, each with distinct clinical profiles.
Type 1 AIP is now regarded as a manifestation of IgG4-related disease, and those affected have tended to be older and to have a high relapse rate. Patients with Type 2 AIP do not experience relapse and tend to be younger. AIP does not affect long-term survival.〔Sah RP, Chari ST, Pannala R, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. ''Gastroenterology''. 2010 Jul;139(1):140-8; PMID 20353791〕
==Signs and symptoms==
AIP is relatively uncommon〔Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB. Clin Gastroenterol Hepatol. 2006 Aug;4(8):1010-6; quiz 934. Epub 2006 Jul 14. PMID 16843735〕 and is characterized by the following features:
# Scleral Icterus (yellow eyes), jaundice (yellow skin) which is usually painless, usually without acute attacks of pancreatitis.
# Relatively mild symptoms, such as minimal weight loss or nausea.
# Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4.
# The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF).
# Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas.
# Diffuse irregular narrowing of the main pancreatic duct, and stenosis of the intrapancreatic bile duct on endoscopic retrograde cholangiopancreatography (ERCP).
# Rare pancreatic calcification or cyst formation.
# Marked responsiveness to treatment with corticosteroids.
AIP is sometimes associated with other autoimmune disorders, most commonly Sjögren's syndrome, primary sclerosing cholangitis (PSC), and inflammatory bowel disease. AIP occurring in association with an autoimmune disorder has been referred to as "secondary" or "syndromic" AIP. Two-thirds of patients present with either obstructive jaundice or a "mass" in the head of the pancreas mimicking carcinoma. It is mandatory to rule out carcinoma prior to making a diagnosis of AIP.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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