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JPTM > Volume 45(5); 2011 > Article
The Korean Journal of Pathology 2011;45(5): 491-497.
doi: https://doi.org/10.4132/KoreanJPathol.2011.45.5.491
Idiopathic Duct Centric Pancreatitis in Korea: A Clinicopathological Study of 14 Cases.
Hyo Jeong Kang, Tae Jun Song, Eunsil Yu, Jihun Kim
1Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. jihunkim@amc.seoul.kr
2Division of Gastroenterology, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje Universitiy College of Medicine, Goyang, Korea.
ABSTRACT
BACKGROUND: Idiopathic duct centric pancreatitis (IDCP) is a subtype of autoimmune pancreatitis (AIP) that is histologically characterized by granulocytic epithelial lesion and scarce IgG4-positive cells. This subtype of AIP has not been documented in Asian countries. METHODS: We reviewed 38 histologically confirmed AIP cases and classified them into lymphoplasmacytic sclerosing pancreatitis (LPSP) and IDCP. Then, clinicopathological characteristics were compared between LPSP and IDCP. RESULTS: Fourteen cases (36.8%) were IDCP. IDCP affected younger patients more than LPSP. IDCP was associated with ulcerative colitis in 35.7% of cases, whereas LPSP was associated with IgG4-related sclerosing diseases such as cholangitis, retroperitoneal fibrosis or sialadenitis in 41.7% of cases. IDCP was microscopically characterized by neutrophilic ductoacinitis with occasional granulocytic epithelial lesions, whereas LPSP was characterized by storiform inflammatory cell-rich fibrosis and obliterative phlebitis. IgG4-positive cells were not detected in any IDCP case but more than 20 IgG4-positive cells per high-power-field were invariably detected in LPSP cases. All patients with IDCP responded dramatically to steroids without recurrence, whereas 33.3% of patients with LPSP developed recurrences. CONCLUSIONS: IDCP is clinicopathologically distinct from LPSP and can be diagnosed when neutrophilic ductoacinitis or granulocytic epithelial lesions are observed in a pancreatic biopsy under the appropriate clinical setting.
Key Words: Autoimmune pancreatitis; Idiopathic duct centric pancreatitis; Granulocytic epithelial lesion; Biopsy, Needle