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Autoimmune pancreatitis (AIP) is a unique form of inflammatory disorders that affect this organ. Histopathology reveals dense infiltration of T lymphocytes and IgG4-positive plasma cells with fibrosis and obliterative phlebitis, termed lymphoplasmacytic sclerosing pancreatitis (LPSP). Various extrapancreatic lesions associated with AIP have similar histological features. Therefore, AIP is currently considered a pancreatic manifestation of an IgG4-related systemic disease. In some cases, there is clinical involvement of only 1 or 2 organs, while in others 3 or 4 are affected. However, precise pathogenetic mechanisms, including the role of IgG4, remain unclear. Stenosis of the bile duct is frequently observed in AIP patients; histologically, IgG4-related sclerosing cholangitis, a bile duct lesion of the systemic disease, is revealed. Stenosis is seen most frequently (70-79%) in the lower bile duct of AIP patients, but this may be due in part to compression caused by pancreatic edema, in addition to biliary wall thickening. 9 When stenosis develops in the hilar or intrahepatic bile duct, the cholangiographic appearance is similar to that of primary sclerosing cholangitis (PSC). PSC is a progressive disease that eventually involves intra-and extra-hepatic bile ducts, and which sometimes leads to liver cirrhosis. The value of steroid therapy is questionable, and liver transplantation is the only effective curative treatment. Since IgG4-related sclerosing cholangitis responds well to steroid therapy, it is necessary to differentiate this from PSC in order to provide the most appropriate treatment regimen.

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2011-09-14   |   1,374 visitas   |   Evalua este artículo 0 valoraciones

Vol. 10 Núm.4. Octubre-Diciembre 2011 Pags. 552-555 Ann Hepatol 2011; 10(4)