Background Patients with Progressive Idiopathic Fibrotic Interstitial Lung Diseases (PIF-ILD) such as idiopathic pulmonary fibrosis have a short disease trajectory and have a similar prognosis to lung cancer patients. They have clear symptom control and quality of life (Qol) needs. The objective of this review was to evaluate the evidence for the use of pharmacological and non-pharmacological methods in improving dyspnoea, other symptoms and Qol for patients with PIF-ILD. In addition we assessed the use of outcome scales and economic evaluation of interventions.

Methods Studies were identified by searching eleven databases, relevant websites and hand searching key journals. Relevant studies were selected, assessed and data extracted independently by two researchers using standardised proformas. Meta-analyses were performed where appropriate and results presented as pooled mean difference with 95%CI. Effect sizes were also calculated where possible. A descriptive summary of other studies has been given.

Results 35 papers with 18 interventions were included. Meta-analyses were only possible for 3 interventions. Meta-analysis showed no significant treatment effect of IFN gamma 1b or sildenafil on 6MWD or dyspnea. A positive treatment effect of pulmonary rehabilitation on 6MWD (effect size (95% CI) 27.4 (4.1, 50.7) p=0.02) was seen. Separate analysis showed a positive effect of pulmonary rehabilitation on dyspnea and a trend towards significant results for pulmonary rehabilitation and sildenafil in improving quality of life. There was weak evidence for the improvement of 6MWD using oxygen, dyspnea using prednisolone, diamorphine, D-pencillamine and colchicine, cough using interferon alpha and thalidomide, anxiety using diamorphine, fatigue using pulmonary rehabilitation and Qol using thalidomide and doxycycline which warrants further research. There were a wide range of outcome scales used and no studies with economic evaluation.

Conclusion There is strong evidence for the use of pulmonary rehabilitation to improve 6MWD and moderate evidence for its use in improving dyspnoea and Qol. In addition, there is moderate evidence for sildenafil in improving Qol. There is weak evidence for a number of other interventions. Further research using economic evaluation and uniform outcome measures is needed.