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IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report
RATIONALE: IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely re...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5728972/ https://www.ncbi.nlm.nih.gov/pubmed/29245357 http://dx.doi.org/10.1097/MD.0000000000009140 |
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author | Chen, Chiu-Fan Chu, Kuo-An Tseng, Yen-Chiang Wu, Chang-Che Lai, Ruay-Sheng |
author_facet | Chen, Chiu-Fan Chu, Kuo-An Tseng, Yen-Chiang Wu, Chang-Che Lai, Ruay-Sheng |
author_sort | Chen, Chiu-Fan |
collection | PubMed |
description | RATIONALE: IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. PATIENT CONCERN: A 43-year-old man had chronic cough and dyspnea on exertion for 4 years. Initial chest radiography showed diffuse interstitial infiltration. Follow-up chest computed tomography 4 years later revealed bilateral diffuse centrilobular nodules with tree-in-bud pattern, bronchial wall thickening, and mediastinal lymph nodes. Bilateral diffuse multifocal ground-glass opacities and mosaic attenuation were also observed. Pulmonary function test revealed mixed restrictive and obstructive ventilatory impairment. DIAGNOSES: Video-assisted thoracoscopic surgery (VATS) lung biopsy showed interstitial fibrosis with lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Serum IgG4 level also showed remarkable elevation. Therefore, IgG4-RLD is confirmed. INTERVENTION: VATS wedge resection of right upper lobe and mediastinal lymph node. OUTCOMES: The patient responded well to steroid and immunosuppression therapy, and was regular followed-up in outpatient clinic. LESSONS: IgG4-RLD should be considered not only in ILD, but also in small airway disease. Serum IgG4 level may be a useful tool for screening. |
format | Online Article Text |
id | pubmed-5728972 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-57289722017-12-20 IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report Chen, Chiu-Fan Chu, Kuo-An Tseng, Yen-Chiang Wu, Chang-Che Lai, Ruay-Sheng Medicine (Baltimore) 6700 RATIONALE: IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. PATIENT CONCERN: A 43-year-old man had chronic cough and dyspnea on exertion for 4 years. Initial chest radiography showed diffuse interstitial infiltration. Follow-up chest computed tomography 4 years later revealed bilateral diffuse centrilobular nodules with tree-in-bud pattern, bronchial wall thickening, and mediastinal lymph nodes. Bilateral diffuse multifocal ground-glass opacities and mosaic attenuation were also observed. Pulmonary function test revealed mixed restrictive and obstructive ventilatory impairment. DIAGNOSES: Video-assisted thoracoscopic surgery (VATS) lung biopsy showed interstitial fibrosis with lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Serum IgG4 level also showed remarkable elevation. Therefore, IgG4-RLD is confirmed. INTERVENTION: VATS wedge resection of right upper lobe and mediastinal lymph node. OUTCOMES: The patient responded well to steroid and immunosuppression therapy, and was regular followed-up in outpatient clinic. LESSONS: IgG4-RLD should be considered not only in ILD, but also in small airway disease. Serum IgG4 level may be a useful tool for screening. Wolters Kluwer Health 2017-12-08 /pmc/articles/PMC5728972/ /pubmed/29245357 http://dx.doi.org/10.1097/MD.0000000000009140 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 |
spellingShingle | 6700 Chen, Chiu-Fan Chu, Kuo-An Tseng, Yen-Chiang Wu, Chang-Che Lai, Ruay-Sheng IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title | IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title_full | IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title_fullStr | IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title_full_unstemmed | IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title_short | IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report |
title_sort | igg4-related lung disease presenting as interstitial lung disease with bronchiolitis: a case report |
topic | 6700 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5728972/ https://www.ncbi.nlm.nih.gov/pubmed/29245357 http://dx.doi.org/10.1097/MD.0000000000009140 |
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