Intrathoracic Manifestations of IgG4-Related Disease
Intrathoracic involvement with IgG4-related disease (IgG4-RD) is frequently overlooked in IgG4-related disease patients. In this article we review the intrathoracic findings of IgG4-RD which are variable and protean. IgG4-related disease has been reported to affect the lung parenchyma, pleura, media...
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| Format: | Article |
| Language: | English |
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Southwest Respiratory and Critical Care Chronicles
2016-10-01
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| Series: | Southwest Respiratory and Critical Care Chronicles |
| Subjects: | |
| Online Access: | http://pulmonarychronicles.com/index.php/pulmonarychronicles/article/view/324 |
| _version_ | 1819174955568332800 |
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| author | Sian Yik Lim Deepa Panikkath |
| author_facet | Sian Yik Lim Deepa Panikkath |
| author_sort | Sian Yik Lim |
| collection | DOAJ |
| description | Intrathoracic involvement with IgG4-related disease (IgG4-RD) is frequently overlooked in IgG4-related disease patients. In this article we review the intrathoracic findings of IgG4-RD which are variable and protean. IgG4-related disease has been reported to affect the lung parenchyma, pleura, mediastinal/hilar lymph nodes, vasculature, and pericardium within the thorax. Mediastinal and hilar lymphadenopathy is the most common intrathoracic manifestation of IgG4-RD. Four main patterns of pulmonary disease have been described, including the solid nodular type, the bronchovascular type, the alveolar interstitial type, and the round shaped ground glass type. When feasible, a biopsy should be obtained to confirm the diagnosis. Most lesions show characteristic pathologic findings of IgG4-RD: dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. While this helps establish the diagnosis, the interpretation of pathology findings in the clinical context is key in making an accurate diagnosis. Mimickers of IgG4-RD should be ruled out, before making a diagnosis. The intrathoracic findings of IgG4-RD can be treated effectively with prednisone, but may require additional immunosuppressive therapies, including rituximab. |
| first_indexed | 2024-12-22T20:47:12Z |
| format | Article |
| id | doaj.art-6f0e71a1c5bd43b0b1bb76044add5f1d |
| institution | Directory Open Access Journal |
| issn | 2325-9205 |
| language | English |
| last_indexed | 2024-12-22T20:47:12Z |
| publishDate | 2016-10-01 |
| publisher | Southwest Respiratory and Critical Care Chronicles |
| record_format | Article |
| series | Southwest Respiratory and Critical Care Chronicles |
| spelling | doaj.art-6f0e71a1c5bd43b0b1bb76044add5f1d2022-12-21T18:13:11ZengSouthwest Respiratory and Critical Care ChroniclesSouthwest Respiratory and Critical Care Chronicles2325-92052016-10-014163339285Intrathoracic Manifestations of IgG4-Related DiseaseSian Yik LimDeepa PanikkathIntrathoracic involvement with IgG4-related disease (IgG4-RD) is frequently overlooked in IgG4-related disease patients. In this article we review the intrathoracic findings of IgG4-RD which are variable and protean. IgG4-related disease has been reported to affect the lung parenchyma, pleura, mediastinal/hilar lymph nodes, vasculature, and pericardium within the thorax. Mediastinal and hilar lymphadenopathy is the most common intrathoracic manifestation of IgG4-RD. Four main patterns of pulmonary disease have been described, including the solid nodular type, the bronchovascular type, the alveolar interstitial type, and the round shaped ground glass type. When feasible, a biopsy should be obtained to confirm the diagnosis. Most lesions show characteristic pathologic findings of IgG4-RD: dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. While this helps establish the diagnosis, the interpretation of pathology findings in the clinical context is key in making an accurate diagnosis. Mimickers of IgG4-RD should be ruled out, before making a diagnosis. The intrathoracic findings of IgG4-RD can be treated effectively with prednisone, but may require additional immunosuppressive therapies, including rituximab.http://pulmonarychronicles.com/index.php/pulmonarychronicles/article/view/324IgG4 related-diseaseIgG4 lung diseasepleural diseasepulmonary disease |
| spellingShingle | Sian Yik Lim Deepa Panikkath Intrathoracic Manifestations of IgG4-Related Disease Southwest Respiratory and Critical Care Chronicles IgG4 related-disease IgG4 lung disease pleural disease pulmonary disease |
| title | Intrathoracic Manifestations of IgG4-Related Disease |
| title_full | Intrathoracic Manifestations of IgG4-Related Disease |
| title_fullStr | Intrathoracic Manifestations of IgG4-Related Disease |
| title_full_unstemmed | Intrathoracic Manifestations of IgG4-Related Disease |
| title_short | Intrathoracic Manifestations of IgG4-Related Disease |
| title_sort | intrathoracic manifestations of igg4 related disease |
| topic | IgG4 related-disease IgG4 lung disease pleural disease pulmonary disease |
| url | http://pulmonarychronicles.com/index.php/pulmonarychronicles/article/view/324 |
| work_keys_str_mv | AT sianyiklim intrathoracicmanifestationsofigg4relateddisease AT deepapanikkath intrathoracicmanifestationsofigg4relateddisease |