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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Main Authors: Hammad Bhatti, Ankur Girdhar, Faisal Usman, James Cury, Abubakr Bajwa
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2013-01-01
Series:Annals of Thoracic Medicine
Subjects:
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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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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