Autoimmune pancreatitis (AIP) is now a well-defined disease of the pancreas that differs clinically, radiologically, and therapeutically from all the other inflammatory diseases of the pancreas. It has been recently histologically classified in type 1 and type 2 AIPs, not different at imaging, but with a better clinical outcome in type 2 compared to type 1. From a clinical point of view, the key point in Italy is to differentiate focal form of the disease from pancreatic cancer. The Italian diagnostic algorithm is therefore addressed to exclude cancer in this setting and, therefore, to safely use steroids. In diffuse form of the disease, the clinical problem is to differentiate AIP from acute nonnecrotizing pancreatitis and to exclude neoplasia (cholangiocarcinoma or lymphoma) in some cases. In this chapter, we report the clinical profile of an Italian series of patients with AIP and highlight the main radiological and pathological findings.
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