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

Wim A Wuyts, Marlies Wijsenbeek, Benjamin Bondue, Demosthenes Bouros, Paul Bresser, Carlos Robalo Cordeiro, Ole Hilberg, Jesper Magnusson, Effrosyni D Manali, António Morais, Spyridon Papiris, Saher Shaker, Marcel Veltkamp, Elisabeth Bendstrup

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.

OriginalsprogEngelsk
TidsskriftRespiration; international review of thoracic diseases
Vol/bind99
Udgave nummer1
Sider (fra-til)73-82
Antal sider10
ISSN0025-7931
DOI
StatusUdgivet - 13 dec. 2019

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