The efficacy of treatment for systemic sclerosis interstitial lung disease: results from a meta-analysis.
BACKGROUND
This meta-analysis examined all randomized controlled trials (RCTs) comparing pharmacotherapy for systemic sclerosis (SSc)-interstitial lung disease (ILD) with placebo or alternative drugs on pulmonary function tests (PFTs), quality of life, dyspnea, skin thickness, and adverse events.


METHODS
Forty studies were identified, and from these 2 RCTs examining cyclophosphamide and 1 examining bosentan met inclusion criteria (studies had to be randomized, have at least 20 SSc-ILD patients, and have PFTs as primary outcomes).


RESULTS
Differences between groups for change of PFT scores between baseline and 12 months were not significant when the 3 trials were combined. The treatment effect of cyclophosphamide versus placebo on forced vital capacity revealed a mean difference of 3.30% (95% confidence interval, 0.06-6.54). Diffusing capacity and total lung capacity did not change.


CONCLUSIONS
This conservative yet significant effect of cyclophosphamide demonstrates the need for further investigation of its effectiveness on patient-important outcomes such as dyspnea and quality of life, which could not be evaluated by this meta-analysis. It may be that studies in SSc-ILD need outcomes that are more sensitive to change.
Broad
Kathleen
K
Dalhousie University, Halifax, Nova Scotia, Canada.
Pope
Janet E
JE
D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D016032:Randomized Controlled Trials as Topic; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome; D014797:Vital Capacity / Q000502:physiology
D003520:Cyclophosphamide