Autoimmune form of chronic pancreatitis and lgG4 positive mastitis

Abstract

The autoimmune form of pancreatitis (AIP) is frequently linked to the simultaneous impairment of other bodily organs. This applies particularly to the 1st subtype of the AIP form of lymphoplasmatic sclerosing pancreatitis (LPSP). This type of autoimmune pancreatitis is accompanied by concurrent sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, sicca syndrome and other ultrapancreatic lesions. A typical symptom is a high level of lgG4 immunoglobulin in blood serum and tissues. In our report, and for the first time in specialist literature, we describe the current findings of AIP (according to Asian criteria) and lgG4 positive mastitis, histologically verified in a patient with previously diagnosed Mikulicz syndrome. The effects of corticoid therapy supported the diagnosis of AIP and simultaneously led to the eradication of recurrent mastitis. Our findings confirm the fact that we are currently increasingly confronted with a group of multi-system autoimmune diseases, including autoimmune pancreatitis and its extrapancreatic symptoms. Timely diagnosis and the correct therapy can be effective against a high percentage of cases.