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( Petr Dite ),( Jan Trna ),( Zdenek Kinkor ),( Ivo Novotny ),( Jan Lata ),( Bohuslav Kianioka ),( Marketa Hermanova ) The Editorial Office of Gut and Liver 2013 Gut and Liver Vol.7 No.5
Autoimmune pancreatitis (AIP) type 1 is commonly associ-ated with simultaneous involvement of extrapancreatic organs. Sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, Sjogren syndrome, and other extrapancreatic lesions are often observed concurrently with AIP. High levels of im-munoglobulin G4 (IgG4) in the blood serum and affected tis-sues are typical of this diagnostic entity. We describe a case report of a 58-year-old female with findings of AIP (according to Asian criteria), IgG4-positive mastitis, and histologically verified Mikulicz syndrome. The effect of corticoid therapy supported the diagnosis of AIP and simultaneously led to the eradication of recurrent mastitis. To the best of our knowl-edge, this is the first reported case of concurrent findings of AIP and IgG4 mastitis. Our case report supports the concept of systemic IgG4 syndrome with multisystem involvement. Timely diagnosis and appropriate therapy can be effective in a high percentage of patients. (Gut Liver 2013;7:621-624)