Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease
Journal of Pathology and Translational Medicine
;
: 300-305, 2016.
Article
in English
| WPRIM
| ID: wpr-32743
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreas
/
Plasma Cells
/
Autoimmune Diseases
/
Bile Ducts
/
Biliary Tract
/
Fibrosis
/
Cholangitis
/
Follow-Up Studies
/
Cholangiocarcinoma
/
Lymph Nodes
Type of study:
Observational study
/
Prognostic study
Limits:
Humans
/
Male
Language:
English
Journal:
Journal of Pathology and Translational Medicine
Year:
2016
Type:
Article
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