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Published on: July 30, 2011
Clinicopathological Features of Type 2 Autoimmune Pancreatitis in Japan: Results of a Multicenter Survey
Kenji Notohara1, Isao Nishimori, Nobumasa Mizuno
1From the *Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki; †Nishimori Clinic, Sakawa, Kochi; ‡Department of Gastroenterology, Aichi Cancer Center Hospital, Nagoya; §Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka; ∥Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka; ¶Center for Health, Safety, and Environmental Management, Shinshu University, Matsumoto; #Division of Hepato-Biliary-Pancreatic Surgery, Tohoku University Graduate School of Medicine, Sendai; **Department of Gastroenterology, Kitakyushu General Hospital, Kitakyushu; and †† Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Objective:
The aim of the study was to clarify clinicopathological features of type 2 autoimmune pancreatitis (AIP) in Japan; a multicenter survey was carried out.
Methods:
The first screening collected patients with pancreatitis whose pancreatic tissue samples were available and who fulfilled at least 1 of the following 3 criteria as possible type 2 AIP: (1) histological presence of granulocytic epithelial lesion, (2) age of 50 years or younger, and (3) association of ulcerative colitis, Sjogren syndrome, and/or primary biliary cirrhosis. Patients with histologically confirmed type 1 AIP were also collected as a control. Clinical information was gathered by questionnaire.
Results:
A histological re-evaluation identified 8 patients with type 2 AIP and 20 with type 1 AIP. Three of the latter had intralobular neutrophilic infiltration. Factors more frequent in type 2 included age younger than 40 years, abdominal pain, and elevation of serum amylase and lipase, whereas patients with type 1 more frequently showed jaundice, elevated serum IgG and IgG4, presence of autoantibodies, association of IgG4-related disease, sclerosing cholangitis and diabetes mellitus, and imaging findings of intrapancreatic biliary stenosis and extrapancreatic biliary dilatation.
Conclusions:
The clinical features of type 2 AIP in Japan were similar to those of western countries. Intralobular neutrophilic infiltration in type 1 is a potential pitfall, especially in the biopsy-based diagnosis.
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