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IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma
IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among th...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983604/ https://www.ncbi.nlm.nih.gov/pubmed/33776680 http://dx.doi.org/10.1159/000513029 |
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author | Minh Thong, Pham Dang Luu, Vu Tra My, Thieu-Thi Xuan Hien, Nguyen Anh Tuan, Tran Minh Duc, Nguyen |
author_facet | Minh Thong, Pham Dang Luu, Vu Tra My, Thieu-Thi Xuan Hien, Nguyen Anh Tuan, Tran Minh Duc, Nguyen |
author_sort | Minh Thong, Pham |
collection | PubMed |
description | IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC. |
format | Online Article Text |
id | pubmed-7983604 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-79836042021-03-26 IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma Minh Thong, Pham Dang Luu, Vu Tra My, Thieu-Thi Xuan Hien, Nguyen Anh Tuan, Tran Minh Duc, Nguyen Case Rep Oncol Case Report IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC. S. Karger AG 2021-02-18 /pmc/articles/PMC7983604/ /pubmed/33776680 http://dx.doi.org/10.1159/000513029 Text en Copyright © 2021 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Case Report Minh Thong, Pham Dang Luu, Vu Tra My, Thieu-Thi Xuan Hien, Nguyen Anh Tuan, Tran Minh Duc, Nguyen IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title_full | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title_fullStr | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title_full_unstemmed | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title_short | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
title_sort | igg4-related sclerosing cholangitis mimicking cholangiocarcinoma |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983604/ https://www.ncbi.nlm.nih.gov/pubmed/33776680 http://dx.doi.org/10.1159/000513029 |
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