Criteria:
(1) both of the following (imaging findings)
(1a) enlarged pancreas (focal, segmental, diffuse) with or without (mass AND/OR hypoattentuation rim) on CT or MRI
(1b) pancreatic ductal narrowing (focal, segmental, diffuse) often with stenosis of the bile duct on MRCP or ERCP
(2) one or both of the following (serologic findings)
(2a) elevated serum IgG or IgG4
(2b) presence of autoantibodies
(3) one or both of the following (histopathologic findings of lesion)
(3a) lymphoplasmacytic infiltrate and fibrosis, often with obliterative phlebitis
(3b) > 10 IgG4 positivie plasma cells per high power field (“abundant”)
(4) response to corticosteroids, with complete resolution or marked improvement
(5) both of the following
(5a) unexplained pancreatic disease
(5b) characteristic pancreatic histology
(6) both of the following
(6a) other organ involvement AND/OR serologic abnormalities
(6b) abnormal imaging findings suggesting chronic pancreatitis AND negative workup for known etiologies
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Diagnosis
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Requirement
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definite
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criterion 1 AND (one or more criteria II to IV)
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probable
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criterion 5 OR criterion 6
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