Autoimmune pancreatitis

Abstract

Autoimmune pancreatitis is a rare form of chronic pancreatitis. Its clinical relevance, however, cannot be dismissed, as it can be difficult to distinguish autoimmune pancreatitis from malignant pancreatic cancer and in contrast with the majority of chronic pancreatitis forms it can be efficiently treated, even complete remission can be achieved on steroid therapy. The clinical picture of autoimmune pancreatitis is not characteristic, obstructive jaundice, abdominal pain, weight loss are frequently observed. Imaging studies often show diffuse pancreas enlargement and irregular narrowing of the main pancreatic duct. Elevated serum IgG4 immunoglobulin concentrations, some autoantibodies and the presence of IgG4 positive immune cells were observed in addition to other histological features. Apart from pancreatic manifestations, other organs may also be affected, thus associations with sclerosing cholangitis, sialoadenitis, retroperitoneal fibrosis, Riedel thyroiditis and inflammatory bowel diseases have been described. Based on these findings, autoimmune pancreatitis should be regarded as a systemic disease, as a manifestation of systemic IgG4-related sclerosing disease.