title: Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature.
abstract: 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.
authors.affiliation: Department of Palliative Medicine, Royal Marsden and Royal Brompton NHS Foundation Trusts, London SW3 6JJ, UK. sabrina.bajwah@kcl.ac.uk
authors.forename: Sabrina
authors.initials: S
authors.lastname: Bajwah
authors.forename: Joy R
authors.initials: JR
authors.lastname: Ross
authors.forename: Janet L
authors.initials: JL
authors.lastname: Peacock
authors.forename: Irene J
authors.initials: IJ
authors.lastname: Higginson
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.forename: Amit Suresh
authors.initials: AS
authors.lastname: Patel
authors.forename: Jonathan
authors.initials: J
authors.lastname: Koffman
authors.forename: Julia
authors.initials: J
authors.lastname: Riley
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D003371:Cough / Q000188:drug therapy / Q000209:etiology; D004417:Dyspnea / Q000188:drug therapy / Q000209:etiology; D005080:Exercise Test; D005355:Fibrosis; D005938:Glucocorticoids / Q000627:therapeutic use; D003932:Heroin / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000188:drug therapy* / Q000534:rehabilitation*; D009294:Narcotics / Q000627:therapeutic use; D010102:Oxygen Inhalation Therapy; D010879:Piperazines / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D011728:Pyridones / Q000627:therapeutic use; D011788:Quality of Life; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use; D013792:Thalidomide / Q000627:therapeutic use; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D007155:Immunologic Factors; D009294:Narcotics; D010879:Piperazines; D011687:Purines; D011728:Pyridones; D013450:Sulfones; D050257:Tubulin Modulators; D013792:Thalidomide; D003932:Heroin; D011239:Prednisolone; D000068677:Sildenafil Citrate; C093844:pirfenidone; D003078:Colchicine