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Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease

Authors
  • Wuyts, Wim A.
  • Wijsenbeek, Marlies
  • Bondue, Benjamin
  • Bouros, Demosthenes
  • Bresser, Paul
  • Robalo Cordeiro, Carlos
  • Hilberg, Ole
  • Magnusson, Jesper
  • Manali, Effrosyni D.
  • Morais, António
  • Papiris, Spyridon
  • Shaker, Saher
  • Veltkamp, Marcel
  • Bendstrup, Elisabeth
Type
Published Article
Journal
Respiration
Publisher
S. Karger AG
Publication Date
Dec 12, 2019
Volume
99
Issue
1
Pages
73–82
Identifiers
DOI: 10.1159/000504763
PMID: 31830755
Source
Karger
Keywords
License
Green
External links

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease that is, by definition, progressive. Progression of IPF is reflected by a decline in lung function, worsening of dyspnea and exercise capacity, and deterioration in health-related quality of life. In the short term, the course of disease for an individual patient is impossible to predict. A period of relative stability in forced vital capacity (FVC) does not mean that FVC will remain stable in the near future. Frequent monitoring using multiple assessments, not limited to pulmonary function tests, is important to evaluate disease progression in individual patients and ensure that patients are offered appropriate care. Optimal management of IPF requires a multidimensional approach, including both pharmacological therapy to slow decline in lung function and supportive care to preserve patients’ quality of life.

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