OBJECTIVES The ideal diagnostic criteria of autoimmune pancreatitis (AIP) are still challenging. Therefore, we investigated the clinical features of AIP in Korea and assessed the clinical use of new Korean diagnostic criteria.

METHODS We reviewed 67 patients with AIP enrolled in 16 hospitals via a multicenter study. The diagnosis was confirmed according to the Korean diagnostic criteria that included pancreatic imaging, laboratory findings, histopathology, and response to steroid.

RESULTS Mean age of the patients was 56 years, and 73% were men. Obstructive jaundice (52%) was the most common symptom, and 14 patients (21%) had other organ involvement. Fifty-four patients (81%) revealed diffuse swelling of the pancreas. Either immunoglobulin (Ig)G or IgG4 was elevated in 76%. According to the Korean criteria, 65 patients had definite diagnostic criteria, and 2 patients had probable criteria. Fifteen patients were fulfilled with image, serological, and histopathologic criteria, and 4 patients could be diagnosed with image and steroid responsiveness. Ten patients experienced recurrent attacks of AIP during the mean 20-month follow-up.

CONCLUSIONS Among 67 cases of AIP, either IgG or IgG4 was elevated in 76% of patients, and 14 patients (21%) had other organ involvement. New Korean diagnostic criteria are useful for diagnosis of AIP.