Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature

Thorax. 2013 Sep;68(9):867-79. doi: 10.1136/thoraxjnl-2012-202040. Epub 2012 Dec 1.


Background: Patients with fibrotic interstitial lung disease have symptom control and quality of life (QoL) needs. This review aims to evaluate the evidence for the use of interventions in improving dyspnoea, other symptoms and QoL.

Methods: Eleven databases, relevant websites and key journals were hand-searched. Studies were assessed and data extracted independently by two researchers using standardised proformas. Meta-analyses were performed where possible with 95% CI.

Results: 34 papers with 19 interventions in 3635 patients were included. Meta-analyses showed no significant effect of interferon γ-1b or sildenafil on 6-minute walking distance (6MWD) or dyspnoea. Pulmonary rehabilitation and pirfenidone had a positive effect on 6MWD (mean difference (95% CI) 27.4 (4.1 to 50.7)) and 24.0 (4.3 to 43.7), respectively), and pulmonary rehabilitation had a mixed effect on dyspnoea. Both pulmonary rehabilitation and sildenafil showed a trend towards significance in improving QoL. There was weak evidence for the improvement of 6MWD using oxygen; dyspnoea using prednisolone, diamorphine, D-pencillamine and colchicine; cough using interferon α and thalidomide; anxiety using diamorphine; fatigue using pulmonary rehabilitation; and QoL using thalidomide and doxycycline. A wide range of outcome scales was used and there were no studies with economic evaluation.

Conclusions: There is strong evidence for the use of pulmonary rehabilitation and pirfenidone to improve 6MWD and moderate evidence for the use of sildenafil and pulmonary rehabilitation to improve QoL. Future recommendations for research would include careful consideration of the dichotomy of radical and palliative treatments when deciding on how symptom and QoL outcome measures are used and data presented.

Keywords: Interstitial Fibrosis; Palliative Care; Pulmonary Rehabilitation.

Publication types

  • Meta-Analysis
  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Anti-Bacterial Agents / therapeutic use
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Colchicine / therapeutic use
  • Cough / drug therapy
  • Cough / etiology
  • Dyspnea / drug therapy
  • Dyspnea / etiology
  • Exercise Test
  • Fibrosis
  • Glucocorticoids / therapeutic use
  • Heroin / therapeutic use
  • Humans
  • Immunologic Factors / therapeutic use
  • Lung / pathology
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / rehabilitation*
  • Narcotics / therapeutic use
  • Oxygen Inhalation Therapy
  • Piperazines / therapeutic use
  • Prednisolone / therapeutic use
  • Purines / therapeutic use
  • Pyridones / therapeutic use
  • Quality of Life*
  • Sildenafil Citrate
  • Sulfones / therapeutic use
  • Thalidomide / therapeutic use
  • Tubulin Modulators / therapeutic use


  • Anti-Bacterial Agents
  • Anti-Inflammatory Agents, Non-Steroidal
  • Glucocorticoids
  • Immunologic Factors
  • Narcotics
  • Piperazines
  • Purines
  • Pyridones
  • Sulfones
  • Tubulin Modulators
  • Thalidomide
  • Heroin
  • Prednisolone
  • Sildenafil Citrate
  • pirfenidone
  • Colchicine