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...
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| Veröffentlicht in: | Case Reports in Oncology 2021-01, Vol.14 (1), p.39-46 |
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| creator | Minh Thong, Pham Dang Luu, Vu Tra My, Thieu-Thi Xuan Hien, Nguyen Anh Tuan, Tran Minh Duc, Nguyen |
| 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. |
| doi_str_mv | 10.1159/000513029 |
| format | Article |
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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.</description><identifier>ISSN: 1662-6575</identifier><identifier>EISSN: 1662-6575</identifier><identifier>DOI: 10.1159/000513029</identifier><identifier>PMID: 33776680</identifier><language>eng</language><publisher>Basel, Switzerland: S. Karger AG</publisher><subject>Abdomen ; Antibodies ; Bile ducts ; biliary dilatation ; Biopsy ; Case Report ; Case reports ; Case studies ; Cholangiocarcinoma ; Cholangitis ; Diagnosis ; Health aspects ; igg4-related disease ; igg4-related sclerosing cholangitis ; Immunoglobulin G ; Immunologic diseases ; Inflammatory diseases ; Kidneys ; Magnetic resonance imaging ; Pancreatitis ; Patients ; Steroids ; Ultrasonic imaging</subject><ispartof>Case Reports in Oncology, 2021-01, Vol.14 (1), p.39-46</ispartof><rights>2021 The Author(s). Published by S. Karger AG, Basel</rights><rights>Copyright © 2021 by S. Karger AG, Basel.</rights><rights>COPYRIGHT 2021 S. Karger AG</rights><rights>2021 The Author(s). Published by S. Karger AG, Basel . This work is licensed under the Creative Commons Attribution – Non-Commercial License http://creativecommons.org/licenses/by-nc/3.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Copyright © 2021 by S. Karger AG, Basel 2021</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c552t-84e5f9d2ed5837eccd1dabb08466722fa1945b8074bcf746b3f307a496bae93b3</citedby><cites>FETCH-LOGICAL-c552t-84e5f9d2ed5837eccd1dabb08466722fa1945b8074bcf746b3f307a496bae93b3</cites><orcidid>0000-0001-5411-1492</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983604/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983604/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,728,781,785,865,886,2103,27640,27929,27930,53796,53798</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33776680$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Minh Thong, Pham</creatorcontrib><creatorcontrib>Dang Luu, Vu</creatorcontrib><creatorcontrib>Tra My, Thieu-Thi</creatorcontrib><creatorcontrib>Xuan Hien, Nguyen</creatorcontrib><creatorcontrib>Anh Tuan, Tran</creatorcontrib><creatorcontrib>Minh Duc, Nguyen</creatorcontrib><title>IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma</title><title>Case Reports in Oncology</title><addtitle>Case Rep Oncol</addtitle><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.</description><subject>Abdomen</subject><subject>Antibodies</subject><subject>Bile ducts</subject><subject>biliary dilatation</subject><subject>Biopsy</subject><subject>Case Report</subject><subject>Case reports</subject><subject>Case studies</subject><subject>Cholangiocarcinoma</subject><subject>Cholangitis</subject><subject>Diagnosis</subject><subject>Health aspects</subject><subject>igg4-related disease</subject><subject>igg4-related sclerosing cholangitis</subject><subject>Immunoglobulin G</subject><subject>Immunologic diseases</subject><subject>Inflammatory diseases</subject><subject>Kidneys</subject><subject>Magnetic resonance imaging</subject><subject>Pancreatitis</subject><subject>Patients</subject><subject>Steroids</subject><subject>Ultrasonic imaging</subject><issn>1662-6575</issn><issn>1662-6575</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>M--</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DOA</sourceid><recordid>eNptkUtr3DAUhUVpaR7tovtSBrLKwumV9d6UhiFNB1ICabsWsh6OJh5rKnsC-fdR6tRMoGghcfWdo3t1EPqA4Qxjpj4DAMMEavUKHWLO64ozwV7vnQ_Q0TCsAbhinL1FB4QIwbmEQ_R11V7S6sZ3ZvRu8dN2Pqch9u1ieZs607dxjMPiR9xEe7dfTdZkG_u0Me_Qm2C6wb9_3o_R728Xv5bfq6vry9Xy_KqyjNVjJalnQbnaOyaJ8NY67EzTgKSci7oOBivKGgmCNjYIyhsSCAhDFW-MV6Qhx2g1-bpk1nqb48bkB51M1H8LKbfa5DGWATQuGowDDYECBWdVkEAs5lIEBlTJ4vVl8trumo131vdjNt0L05c3fbzVbbrXoog50GJw8myQ05-dH0a9Trvcl_l1zURJQ3H29MzZRLWmdBX7kIqZLcv58p-p9yGW-jnnIBlRwIrgdBLYEsKQfZhbwqCfktZz0oX9tD_DTP6LtgAfJ-DO5NbnGZj1J_-9Xt5cT4TeukAeAcNat04</recordid><startdate>20210101</startdate><enddate>20210101</enddate><creator>Minh Thong, Pham</creator><creator>Dang Luu, Vu</creator><creator>Tra My, Thieu-Thi</creator><creator>Xuan Hien, Nguyen</creator><creator>Anh Tuan, Tran</creator><creator>Minh Duc, Nguyen</creator><general>S. 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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.</abstract><cop>Basel, Switzerland</cop><pub>S. Karger AG</pub><pmid>33776680</pmid><doi>10.1159/000513029</doi><tpages>8</tpages><orcidid>https://orcid.org/0000-0001-5411-1492</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Case Reports in Oncology, 2021-01, Vol.14 (1), p.39-46 |
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| source | DOAJ Directory of Open Access Journals; Karger Open Access; EZB-FREE-00999 freely available EZB journals; PubMed Central |
| subjects | Abdomen Antibodies Bile ducts biliary dilatation Biopsy Case Report Case reports Case studies Cholangiocarcinoma Cholangitis Diagnosis Health aspects igg4-related disease igg4-related sclerosing cholangitis Immunoglobulin G Immunologic diseases Inflammatory diseases Kidneys Magnetic resonance imaging Pancreatitis Patients Steroids Ultrasonic imaging |
| title | IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma |
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