Open AccessIdiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease
Author(s) -
Wim Wuyts,
Marlies Wijsenbeek,
Benjamin Bondue,
Demosthenes Bouros,
Paul Bresser,
Carlos Robalo Cordeiro,
Ole Hilberg,
Jesper Magnusson,
Effrosyni D. Manali,
António Morais,
Spyridon Papiris,
Saher Burhan Shaker,
Marcel Veltkamp,
Elisabeth Bendstrup
Publication year - 2019
Publication title -
respiration
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.264
H-Index - 81
eISSN - 1423-0356
pISSN - 0025-7931
DOI - 10.1159/000504763
Subject(s) - medicine , idiopathic pulmonary fibrosis , pulmonary function testing , interstitial lung disease , intensive care medicine , quality of life (healthcare) , vital capacity , lung , lung function , disease , pulmonary fibrosis , physical therapy , diffusing capacity , nursing
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.