Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease.
- Author:
Seokhwi KIM
1
;
Hyunsik BAE
;
Misun CHOI
;
Binnari KIM
;
Jin Seok HEO
;
Ho Seong KIM
;
Seung Hee CHOI
;
Kee Taek JANG
Author Information
- Publication Type:Case Report
- Keywords: IgG4-related disease; Bile ducts; Mass-forming; Cholangiocarcinoma
- MeSH: Autoimmune Diseases; Bile Ducts; Biliary Tract; Cholangiocarcinoma; Cholangitis*; Fibrosis; Follow-Up Studies; Humans; Lymph Nodes; Male; Middle Aged; Pancreas; Plasma Cells
- From:Journal of Pathology and Translational Medicine 2016;50(4):300-305
- CountryRepublic of Korea
- Language:English
- Abstract: IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.
