The list of diseases associated with autoantibodies against tissues, cells, or specific autoantigens is growing, and many organs in the body are known to be affected by autoimmune injury. Until recently, the most well-known pancreatic autoimmune disorder was type I diabetes mellitus, where there is selective destruction of beta cells in the islets of Langerhans. Although an autoimmune process affecting the exocrine pancreas was suspected over four decades ago, it is only in the past 10 years or so that autoimmune chronic pancreatitis has been recognized as a distinct entity. Here we review the clinical, serologic, radiologic, and histologic features of autoimmune pancreatitis.
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