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Autoimmune Pancreatitis as a New Clinical Entity (Three Cases of Autoimmune Pancreatitis with Effective Steroid Therapy)

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Abstract

The most common forms of chronic pancreatitisare related to alcohol ingestion, whereas the entity ofnon-alcohol-associated (idiopathic) pancreatitis ispoorly understood. Autoimmunity has been suggested as a possible etiologic factor of idiopathicchronic pancreatitis. A total of 362 Japanese patientsunderwent endoscopic retrograde pancreatography (ERP)for suspected pancreatic disease, and 161 were diagnosed with chronic pancreatitis. Among them, we foundthree cases (1.86% incidence) of unique chronicpancreatitis, in which ERP revealed diffuse narrowing ofthe main pancreatic duct with an irregular wall. We diagnosed these three patients as havingpancreatitis associated with an autoimmune mechanismmorphologically and biochemically and started them onsteroid therapy. The characteristics of the these three patients were as follows:hypergammaglobulinemia, eosinophilia, ultrasonographyshowing hypoehoic diffuse swelling in the pancreas(sausage-like appearance), ERP showing diffuse narrowingof the main pancreatic duct with irregular like thumbprintlike marks,reversible exocrine insufficiency, and positiveanti-carbonic anhydrase II antibody. After one month ofthe treatment with steroids, pancreatitis dramatically improved morphologically and enzymatically.Here we describe these cases of the suspected autoimmunechronic pancreatitis. We must recognize the concept andthe features of autoimmune pancreatitis in order to avoid unnecessary surgery as pancreaticcancer.

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Authors
  1. Tetsuhide Ito
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  2. Itsuro Nakano
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  3. Shujiro Koyanagi
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  4. Toshihiko Miyahara
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  5. Yoshikatsu Migita
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  6. Keiichiro Ogoshi
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  7. Hironori Sakai
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  8. Shizu Matsunaga
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  9. Osamu Yasuda
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  10. Toshihiko Sumii
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  11. Hajime Nawata
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Ito, T., Nakano, I., Koyanagi, S. et al. Autoimmune Pancreatitis as a New Clinical Entity (Three Cases of Autoimmune Pancreatitis with Effective Steroid Therapy). Dig Dis Sci 42, 1458–1468 (1997). https://doi.org/10.1023/A:1018862626221

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  • DOI: https://doi.org/10.1023/A:1018862626221

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