Approach to acute exacerbation of idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial pneumonia with a median survival of 3 years after diagnosis. Acute exacerbation of IPF (AE-IPF) is now identified as a life-threatening complication. It presents as worsening dyspnea with new ground glass opacities superimposed upon a rad...
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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2013-01-01
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Series: | Annals of Thoracic Medicine |
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Online Access: | http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2013;volume=8;issue=2;spage=71;epage=77;aulast=Bhatti |
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author | Hammad Bhatti Ankur Girdhar Faisal Usman James Cury Abubakr Bajwa |
author_facet | Hammad Bhatti Ankur Girdhar Faisal Usman James Cury Abubakr Bajwa |
author_sort | Hammad Bhatti |
collection | DOAJ |
description | Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial pneumonia with a median survival of 3 years after diagnosis. Acute exacerbation of IPF (AE-IPF) is now identified as a life-threatening complication. It presents as worsening dyspnea with new ground glass opacities superimposed upon a radiographic usual interstitial pneumonia (UIP) pattern. It is a diagnosis of exclusion. The prognosis of AE-IPF is poor and treatment strategies lack standardization. In order to rule out any reversible etiology for an acute decompensation of a previously stable IPF patient diagnostic modalities include computerized tomographic angiogram (CTA) coupled with high-resolution computerized tomography (HRCT) imaging of the chest, bronchoalveolar lavage (BAL) and echocardiogram with bubble study. Avoiding risk factors, identifying underlying causes and supportive care are the mainstays of treatment. Anti-inflammatory and immunosuppressant medications have not shown to improve survival in AE-IPF. Most of the patients are managed in a critical care setting with mechanical ventilation. Lung transplantation is a promising option but most institutions are not equipped and not every patient is a candidate. |
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id | doaj.art-ee84c7a4a5da4f3686af131916edb09c |
institution | Directory Open Access Journal |
issn | 1817-1737 1998-3557 |
language | English |
last_indexed | 2024-12-14T00:52:08Z |
publishDate | 2013-01-01 |
publisher | Wolters Kluwer Medknow Publications |
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series | Annals of Thoracic Medicine |
spelling | doaj.art-ee84c7a4a5da4f3686af131916edb09c2022-12-21T23:23:47ZengWolters Kluwer Medknow PublicationsAnnals of Thoracic Medicine1817-17371998-35572013-01-0182717710.4103/1817-1737.109815Approach to acute exacerbation of idiopathic pulmonary fibrosisHammad BhattiAnkur GirdharFaisal UsmanJames CuryAbubakr BajwaIdiopathic pulmonary fibrosis (IPF) is a chronic interstitial pneumonia with a median survival of 3 years after diagnosis. Acute exacerbation of IPF (AE-IPF) is now identified as a life-threatening complication. It presents as worsening dyspnea with new ground glass opacities superimposed upon a radiographic usual interstitial pneumonia (UIP) pattern. It is a diagnosis of exclusion. The prognosis of AE-IPF is poor and treatment strategies lack standardization. In order to rule out any reversible etiology for an acute decompensation of a previously stable IPF patient diagnostic modalities include computerized tomographic angiogram (CTA) coupled with high-resolution computerized tomography (HRCT) imaging of the chest, bronchoalveolar lavage (BAL) and echocardiogram with bubble study. Avoiding risk factors, identifying underlying causes and supportive care are the mainstays of treatment. Anti-inflammatory and immunosuppressant medications have not shown to improve survival in AE-IPF. Most of the patients are managed in a critical care setting with mechanical ventilation. Lung transplantation is a promising option but most institutions are not equipped and not every patient is a candidate.http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2013;volume=8;issue=2;spage=71;epage=77;aulast=BhattiAcute exacerbation of idiopathic pulmonary fibrosisbronchoalveolar lavagechest roentgenogramcomputerized tomographic angiogramhigh resolution computer tomographyidiopathic pulmonary fibrosisusual interstitial pneumonia |
spellingShingle | Hammad Bhatti Ankur Girdhar Faisal Usman James Cury Abubakr Bajwa Approach to acute exacerbation of idiopathic pulmonary fibrosis Annals of Thoracic Medicine Acute exacerbation of idiopathic pulmonary fibrosis bronchoalveolar lavage chest roentgenogram computerized tomographic angiogram high resolution computer tomography idiopathic pulmonary fibrosis usual interstitial pneumonia |
title | Approach to acute exacerbation of idiopathic pulmonary fibrosis |
title_full | Approach to acute exacerbation of idiopathic pulmonary fibrosis |
title_fullStr | Approach to acute exacerbation of idiopathic pulmonary fibrosis |
title_full_unstemmed | Approach to acute exacerbation of idiopathic pulmonary fibrosis |
title_short | Approach to acute exacerbation of idiopathic pulmonary fibrosis |
title_sort | approach to acute exacerbation of idiopathic pulmonary fibrosis |
topic | Acute exacerbation of idiopathic pulmonary fibrosis bronchoalveolar lavage chest roentgenogram computerized tomographic angiogram high resolution computer tomography idiopathic pulmonary fibrosis usual interstitial pneumonia |
url | http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2013;volume=8;issue=2;spage=71;epage=77;aulast=Bhatti |
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