Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

• Published in: Journal of pathology and translational medicine Vol. 50; no. 4; pp. 300 - 305
• Main Authors: Kim, Seokhwi, Bae, Hyunsik, Choi, Misun, Kim, Binnari, Heo, Jin Seok, Kim, Ho Seong, Choi, Seung Hee, Jang, Kee-Taek
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Society of Pathologists, Korean Society for Cytopathology 01.07.2016; The Korean Society of Pathologists and the Korean Society for Cytopathology; Korean Society of Pathologists & the Korean Society for Cytopathology; 대한병리학회
• Subjects: Antigens; Autoimmune diseases; Bile; Bile ducts; Biopsy; Case Study; Cholangiocarcinoma; Family medical history; IgG4-related disease; Immunoglobulins; Laboratories; Liver; Lymphatic system; Mass-forming; Medical imaging; NMR; Nuclear magnetic resonance; Pancreatic cancer; 병리학; IgG4-related disease; Mass-forming; Bile ducts; Cholangiocarcinoma
• ISSN: 2383-7837; 2383-7845
• DOI: 10.4132/jptm.2015.12.01

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.