IgG4 Cholangiopathy
IgG4 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions. Most cases are associated with autoimmune pancreatitis, an important diagnostic clue. Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy is...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | International Journal of Hepatology |
| Online Access: | http://dx.doi.org/10.1155/2012/472376 |
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| _version_ | 1832559207964999680 |
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| author | Yoh Zen Yasuni Nakanuma |
| author_facet | Yoh Zen Yasuni Nakanuma |
| author_sort | Yoh Zen |
| collection | DOAJ |
| description | IgG4 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions. Most cases are associated with autoimmune pancreatitis, an important diagnostic clue. Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy is challenging. Indeed such cases have been treated surgically. IgG4-cholangiopathy should be diagnosed based on serological examinations including serum IgG4 concentrations, radiological features, and histological evidence of IgG4+ plasma cell infiltration. Steroid therapy is very effective even at disease relapse. A Th2-dominant immune response or the activation of regulatory T cells seems to be involved in the underlying immune reaction. It is still unknown why IgG4 levels are specifically elevated in patients with this disease. IgG4 might be secondarily overexpressed by Th2 or regulatory cytokines given the lack of evidence that IgG4 is an autoantibody. |
| format | Article |
| id | doaj-art-535aeaa252324138ac06a8d71530f686 |
| institution | Kabale University |
| issn | 2090-3448 2090-3456 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Hepatology |
| spelling | doaj-art-535aeaa252324138ac06a8d71530f6862025-02-03T01:30:35ZengWileyInternational Journal of Hepatology2090-34482090-34562012-01-01201210.1155/2012/472376472376IgG4 CholangiopathyYoh Zen0Yasuni Nakanuma1Institute of Liver Studies, King’s College Hospital, Denmark Hill, London SE5 9RS, UKDepartment of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa 920-8640, JapanIgG4 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions. Most cases are associated with autoimmune pancreatitis, an important diagnostic clue. Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy is challenging. Indeed such cases have been treated surgically. IgG4-cholangiopathy should be diagnosed based on serological examinations including serum IgG4 concentrations, radiological features, and histological evidence of IgG4+ plasma cell infiltration. Steroid therapy is very effective even at disease relapse. A Th2-dominant immune response or the activation of regulatory T cells seems to be involved in the underlying immune reaction. It is still unknown why IgG4 levels are specifically elevated in patients with this disease. IgG4 might be secondarily overexpressed by Th2 or regulatory cytokines given the lack of evidence that IgG4 is an autoantibody.http://dx.doi.org/10.1155/2012/472376 |
| spellingShingle | Yoh Zen Yasuni Nakanuma IgG4 Cholangiopathy International Journal of Hepatology |
| title | IgG4 Cholangiopathy |
| title_full | IgG4 Cholangiopathy |
| title_fullStr | IgG4 Cholangiopathy |
| title_full_unstemmed | IgG4 Cholangiopathy |
| title_short | IgG4 Cholangiopathy |
| title_sort | igg4 cholangiopathy |
| url | http://dx.doi.org/10.1155/2012/472376 |
| work_keys_str_mv | AT yohzen igg4cholangiopathy AT yasuninakanuma igg4cholangiopathy |