TO THE EDITOR: We read with interest the article by Church et al. (1) on autoimmune pancreatitis (AIP) in the first series from the United Kingdom. They diagnosed 11 patients with AIP and treated them with oral steroid. Altough initial response to steroid therapy was excellent in all patients, six patients (55%) relapsed on steroid reduction or withdrawal, and four of them responded to azathioprine and increased steroid. Because azathioprine therapy may cause acute pancreatitis and requires frequent blood tests for early detection of side effects (2–4), a safe and simple alternative for refractory AIP is needed. AIP is a recently described unique form of chronic pancreatitis characterized by sausage-like diffuse swelling of the pancreas, a diffusely irregular narrowing of the main pancreatic duct, a high serum IgG4 concentration, and a response to oral steroid therapy (5, 6). In unresponsive patients with the biliary stenosis caused by AIP, surgery may be necessary for the relief of symptoms and for differentiation from malignancy (5). Because oral steroid therapy requires a long period for the drug tapering, the biliary stenosis suspected to be caused by AIP but cannot be distinguished from malignancy is not indicated for the therapy (5, 6). Steroid pulse therapy is a well-recognized alternative for refractory autoimmune disorders without steroid tapering. We, therefore, applied the therapy for refractory AIP, resulting in dramatic response for a short period (5, 6). Azathioprine is an effective drug as a maintenance and steroid-sparing agent in autoimmune disorders and organ transplants (2–4). Although azathioprine is widely used in Crohn’s disease, a major drawback is the frequent occurrence of side effects, especially acute pancreatitis(2). A large population-based case-control study found an eight-fold increased relative risk of acute pancreatitis in all users of azathioprine (3). In animal models, azathioprine involves activation of circulating vasoactive mediators, formation of microthrombi, or direct injury of the capillary endothelium, and then deteriorates pancreatic microcirculation, thereby increasing ischemia and acinar cell injury (4). Altough further comparative studies are needed, we believe that azathioprine therapy should be avoided in pancreatic diseases such as AIP, whereas steroid pulse therapy is a more effective alternative for refractory AIP.
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