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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...

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Autores principales: Chen, Chiu-Fan, Chu, Kuo-An, Tseng, Yen-Chiang, Wu, Chang-Che, Lai, Ruay-Sheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
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.
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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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