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

Respiration. 2020;99(1):73-82. doi: 10.1159/000504763. Epub 2019 Dec 12.

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.

Keywords: Interstitial lung disease; Mortality; Nintedanib; Pirfenidone; Therapeutics; Treatment.

Publication types

  • Review

MeSH terms

  • Disease Management
  • Disease Progression
  • Dyspnea / physiopathology
  • Dyspnea / therapy
  • Exercise Tolerance
  • Humans
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Idiopathic Pulmonary Fibrosis / therapy*
  • Indoles / therapeutic use*
  • Oxygen Inhalation Therapy*
  • Patient Education as Topic
  • Patient-Centered Care
  • Practice Guidelines as Topic*
  • Protein Kinase Inhibitors / therapeutic use*
  • Pulmonary Diffusing Capacity
  • Pyridones / therapeutic use*
  • Quality of Life
  • Respiratory Function Tests
  • Terminal Care
  • Vital Capacity
  • Walk Test

Substances

  • Indoles
  • Protein Kinase Inhibitors
  • Pyridones
  • pirfenidone
  • nintedanib