J Pathol Transl Med.  2016 Jul;50(4):300-305. 10.4132/jptm.2015.12.01.

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

• Kim S
• Bae H
• Choi M
• Kim B
• Heo JS
• Kim HS
• Choi SH
• Jang KT

• KMID: 2345553
• DOI: http://doi.org/10.4132/jptm.2015.12.01

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.

Keyword

IgG4-related disease; Bile ducts; Mass-forming; Cholangiocarcinoma

MeSH Terms

Autoimmune Diseases
Bile Ducts
Biliary Tract
Cholangiocarcinoma
Cholangitis*
Fibrosis
Follow-Up Studies
Humans
Lymph Nodes
Male
Middle Aged
Pancreas
Plasma Cells

Figure

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  Fig. 1. Magnetic resonance imaging reveals an enhancing mass lesion which has high signal intensity on diffusion restriction phase (A) and low intensity on apparent diffusion coefficient map phase (B), suspicious for malignancy. (C) The cut section of hilar bile duct shows a relatively well-demarcated mass-forming lesion with a hepatic parenchymal invasion.

• 

  Fig. 2. Histologic examination reveals dense infiltration of lymphocytes and plasma cells (A) and storiform fibrosis (B). Both IgG-immunopositive (C) and IgG4-immunopositive (D) plasma cells are identified by immunohistochemical staining.

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  Fig. 3. (A) Fluorodeoxyglucose (FDG) positron emission tomography–computed tomography, maximum intensity projection image show hypermetabolic lesions involving bilateral supraclavicular, left axillar, mediastinal, pulmonary hilar and retroperitoneal lymph nodes, right liver biliary tract, pancreas, and perisplenic area. (B) After steroid treatment, markedly decreased FDG uptake is noted although uptakes in some lymph nodes are still seen.

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