Background: Patients with idiopathic pulmonary fibrosis have a poor overall prognosis and there are few evidence based drug therapies that reduce mortality.
Objective: This systematic review and meta-analysis aims to assess whether sildenafil reduces mortality, reduces disease progression and the adverse side effects associated with it.
Methods: In this review, randomized controlled studies (RCTs) were retrieved from MEDLINE, Cochrane, and EMBASE. The primary outcome was mortality. The secondary outcomes included change in FVC, acute exacerbations and hospitalizations and adverse drug effects leading to discontinuation. We used an inverse variance random effects meta-analysis method to calculate pooled odds ratio (OR) and standardized mean difference (SMD).
Results: A total of 4 studies were included. Sildenafil probably reduces mortality when compared to placebo or to standard care, [OR 0.63 (0.38,1.03), I2=0%]. Pooled results showed sildenafil does not alter the rate of change of FVC [SMD 0.02 (-0.14,0.18)], or DLCO [SMR -0.01 (-0.18,0.17)], I2=0]. Pooled results showed sildenafil may not reduce the number of hospitalizations or acute exacerbations, [OR 1.06 (0.67,1.67)], I2= 0]. There was no significant difference in drug discontinuation due to adverse effects when comparing sildenafil to the control group, [OR 0.79 (0.56, 1.11)], I2=0].
Conclusion: Sildenafil probably reduces all-cause mortality in IPF patients. More studies need to be done in order to confirm the magnitude and reliability of the point estimate.