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.

Additional Metadata
Keywords Interstitial lung disease, Mortality, Nintedanib, Pirfenidone, Therapeutics, Treatment
Persistent URL dx.doi.org/10.1159/000504763, hdl.handle.net/1765/122924
Journal Respiration (English Edition)
Citation
Wuyts, W.A, Wijsenbeek-lourens, M.S, Bondue, B. (Benjamin), Bouros, D. (Demosthenes), Bresser, P, Robalo Cordeiro, C. (Carlos), … Bendstrup, E. (2019). Idiopathic pulmonary fibrosis: Best practice in monitoring and managing a relentless fibrotic disease. Respiration (English Edition). doi:10.1159/000504763