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Idiopathic Duct Centric Pancreatitis in Korea: A Clinicopathological Study of 14 Cases

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°­È¿Á¤ ( Kang Hyo-Jeong ) 
University of Ulsan College of Medicine Department of Pathology

À¯Àº½Ç ( Yu Eun-Sil ) 
University of Ulsan College of Medicine Department of Pathology
±èÁöÇå ( Kim Ji-Hun ) 
University of Ulsan College of Medicine Department of Pathology
¼ÛÅÂÁØ ( Song Tae-Jun ) 
Inje University Ilsan Paik Hospital Department of Internal Medicine

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.

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Autoimmune pancreatitis;Idiopathic duct centric pancreatitis;Granulocytic epithelial lesion;Biopsy;Needle

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