chronic pancreatitis; autoimmune pancreatitis; immunoglobulines; IgG4; steroids; extrapancreatic lesions; pancreatic biopsy

Autoimmune pancreatitis (AIP) is the specific type of chronic pancreatitis due to autoimmune background and mechanism. The main clinical symptoms of AIP are obstructive jaundice and abdominal discomfort. The typical histological findings are lymphocytes and IgG4 plasma cells infiltration, fibrosis and venulitis within pancreatic gland. Plasma level of IgG4 is usually extremely high. High level IgG4 positive plasma cells in serum, lymphoplasmatic infiltration found on histological staining of pancreatic tissue, "sausage-like" pancreas in ultrasound and CT scans, and response to steroid therapy are crucial for making of diagnosis. Due to its high effectivity in AIP treatment, steroid therapy is the first-line option. The alternative therapy is using immunosuppressants (azathioprine). Recently, there are also first experience in biological therapy already published.