Abstract

Chronic autoimmune pancreatitis is an entity distinct from all other forms of chronic pancreatitis. It is expressed by signs of acute or chronic pancreatitis, sometimes associated with cholestatic jaundice. In imaging, it may appear as diffuse (duct destructive) or pseudotumoral lesions. These 2 aspects are probably different clinical forms of chronic autoimmune pancreatitis. Some autoimmune diseases are associated with chronic autoimmune pancreatitis, but not consistently. One such disease involves a bile disorder very similar to primary sclerosing cholangitis but responsive to corticosteroid treatment. Pancreatitis may be a sign of intestinal inflammatory diseases (and vice versa): testing for Crohn's disease and ulcerative rectocolitis is justified in patients with idiopathic pancreatitis. Chronic autoimmune pancreatitis must be routinely considered in patients with a pancreatic tumor that is for a clinical, epidemiologic, serologic or imaging reason not completely consistent with pancreatic adenocarcinoma. A short corticosteroid therapy (< 4 weeks) is probably less harmful in a patient with pancreatic adenocarcinoma than pancreatectomy (or chemotherapy) in patients with chronic autoimmune pancreatitis. Diagnosis depends on a body of clinical and radiologic evidence. The diagnostic value of serologic markers and especially of autoantibodies must be clarified in the future.

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