Diagnostic criteria for autoimmune chronic pancreatitis revisited

Abstract

Autoimmune chronic pancreatitis (AIP) is increasingly being recognized worldwidely, as knowledge of this entity builds up. Above all, AIP is a very attractive disease to clinicians in terms of its dramatic response to the oral steroid therapy in contrast to ordinary chronic pancreatitis. Although many characteristic findings of AIP have been described, definite diagnostic criteria have not been fully established. In the year 2002, the Japan Pancreas Society published the diagnostic criteria of AIP and many clinicians around the world use these criteria for the diagnosis of AIP. The diagnostic criteria proposed by the Japan Pancreas Society, however, are not completely satisfactory and some groups use their own criteria in reporting AIP. This review discusses several potential limitations of current diagnostic criteria for this increasingly recognized condition. The manuscript is organized to emphasize the need for convening a consensus to develop improved diagnostic criteria.

Figures

Figure 1

Abdominal CT findings. A: Diffusely enlarged pancreas without any calcification or stones in a 59 year-old man. A capsule-like low-density rim can also be seen around the pancreas; B: After steroid therapy, the pancreas returned to its normal size and the rim disappeared.

Figure 2

ERCP findings. A: Diffuse irregular narrowing of more than 2/3 of the entire length of main pancreatic duct noted in the 49 year-old woman; B: After steroid therapy, evidently widening of the main pancreatic duct.

Figure 3

ERCP findings. A: Segmental narrowing (arrows) of the main pancreatic duct noted at the pancreatic head. The extent of irregular narrowing is less than 1/3 of the entire length of main pancreatic duct; B: Direct pancreatogram revealing a normal-appearing main pancreatic duct following steroid therapy.

Figure 4

ERCP findings. A: Segmental irregular narrowing (arrow heads) of the main pancreatic duct that involves the pancreatic head and tail portion is noted in a 53-year-old man; B: After steroid therapy, the narrowing sites on preceding ERCP were resolved. Biliary stenting for narrowing of intrapancreatic common bile duct is noted.