Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease

Interstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may or may not progress over time. I...

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Main Authors: Kristin Berger, Robert J. Kaner
Format: Article
Language:English
Published: MDPI AG 2023-02-01
Series:Life
Subjects:
Online Access:https://www.mdpi.com/2075-1729/13/3/599
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author Kristin Berger
Robert J. Kaner
author_facet Kristin Berger
Robert J. Kaner
author_sort Kristin Berger
collection DOAJ
description Interstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may or may not progress over time. Idiopathic pulmonary fibrosis is the prototypical, progressive fibrotic interstitial lung disease, which can lead to worsening hypoxemic respiratory failure and mortality within a number of years from the time of diagnosis. The importance of an accurate and timely diagnosis of interstitial lung diseases, which is needed to inform prognosis and guide clinical management, cannot be overemphasized. Developing a consensus diagnosis requires the incorporation of a variety of factors by a multidisciplinary team, which then may or may not determine a need for tissue sampling. Clinical management can be challenging given the heterogeneity of disease behavior and the paucity of controlled trials to guide decision making. This review addresses current paradigms and recent updates in the diagnosis and pharmacologic management of these fibrotic interstitial lung diseases.
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spelling doaj.art-1c1137a89f414f96b38b96f7a47b17762023-11-17T12:09:45ZengMDPI AGLife2075-17292023-02-0113359910.3390/life13030599Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung DiseaseKristin Berger0Robert J. Kaner1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY 10021, USADivision of Pulmonary and Critical Care Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY 10021, USAInterstitial lung disease is an umbrella term that encompasses a spectrum of parenchymal lung pathologies affecting the gas exchanging part of the lung. While many of these disease entities are not fibrotic in nature, a number can lead to pulmonary fibrosis which may or may not progress over time. Idiopathic pulmonary fibrosis is the prototypical, progressive fibrotic interstitial lung disease, which can lead to worsening hypoxemic respiratory failure and mortality within a number of years from the time of diagnosis. The importance of an accurate and timely diagnosis of interstitial lung diseases, which is needed to inform prognosis and guide clinical management, cannot be overemphasized. Developing a consensus diagnosis requires the incorporation of a variety of factors by a multidisciplinary team, which then may or may not determine a need for tissue sampling. Clinical management can be challenging given the heterogeneity of disease behavior and the paucity of controlled trials to guide decision making. This review addresses current paradigms and recent updates in the diagnosis and pharmacologic management of these fibrotic interstitial lung diseases.https://www.mdpi.com/2075-1729/13/3/599pulmonary fibrosisinterstitial lung diseasedrug therapydiagnostic techniquesprocedures
spellingShingle Kristin Berger
Robert J. Kaner
Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
Life
pulmonary fibrosis
interstitial lung disease
drug therapy
diagnostic techniques
procedures
title Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
title_full Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
title_fullStr Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
title_full_unstemmed Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
title_short Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease
title_sort diagnosis and pharmacologic management of fibrotic interstitial lung disease
topic pulmonary fibrosis
interstitial lung disease
drug therapy
diagnostic techniques
procedures
url https://www.mdpi.com/2075-1729/13/3/599
work_keys_str_mv AT kristinberger diagnosisandpharmacologicmanagementoffibroticinterstitiallungdisease
AT robertjkaner diagnosisandpharmacologicmanagementoffibroticinterstitiallungdisease