Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP) is a relatively new syndrome of clinical and histologic findings and has also been described as 'lymphoplasmocytic sclerosing pancreatitis with cholangitis', 'nonalcoholic duct-destructive chronic pancreatitis' and 'chronic sclerosing pancreatitis'. A number of features can be found, including hypergammaglobulinemia, elevation of serum IgG4, IgG4-containing immune complexes and a number of antibodies, such as antinuclear antibodies, antibodies against lactoferrin, carbonic anhydrase type II, and rheumatoid factors. Histological features include fibrosis with lym-phoplasmacytic infiltration of interlobular ducts. The majority of lymphocytes are CD8+ and CD4+ T-lympho-cytes, while B-lymphocytes are less frequent. In general, diagnosis of AIP is established by clinical signs and laboratory and morphological findings. An association with other autoimmune disease such as Sjogren-syndrome, primary biliary cirrhosis, primary sclerosing cholangitis, Crohn's disease or ulcerating colitis, systemic lupus ery-thematosus, and retroperitoneal fibrosis is found in a third of the cases.

At imaging, a focal ('mass-forming') or diffuse ('sausage-like') enlargement of the pancreas may be present. In contrast-enhanced studies, peripancreatic nodular or rim-like enhancement can be appreciated. Focal AIP of the head that involves the pancreatic and distal common bile duct needs to be differentiated from pancreatic carcinoma, necessitating biopsy proof.

Most patients appear to respond to steroid treatment according to their symptoms, and laboratory and morphological findings [33-38].

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