Abstract
Background: There is a paucity of head-to-head comparisons of the efficacy and harms of pharmacological treatments for systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: We conducted a network meta-analysis (NMA) in order to compare the effects of different treatments with the placebo on change in forced vital capacity (FVC), change in diffusion lung capacity for CO (DLCO), serious adverse events (SAEs), discontinuation for adverse events and mortality in SSc-ILD. Standardized mean difference (SMD) and log odds ratio were estimated using NMA with fixed effects. Results: Nine randomized clinical trials (926 participants) comparing eight interventions and the placebo for an average follow-up of one year were included. Compared to the placebo, only rituximab significantly reduced FVC decline (SMD (95% CI) = 1.00 (0.39 to 1.61)). Suitable data on FVC outcome for nintedanib were not available for the analysis. No treatments influenced DLCO. Safety and mortality were also not different across treatments and the placebo, although there were few reported events. Cyclophosphamide and pomalidomide were less tolerated than the placebo, mycophenolate, and nintedanib. Conclusion: Only rituximab significantly reduced lung function decline compared to the placebo. However, direct head-to-head comparison studies are required to confirm these findings and to better determine the safety profile of various treatments.
Highlights
Systemic sclerosis (SSc) is an immune-mediated connective tissue disease (CTD) characterized by extensive fibrosis of the skin and internal organs and vasculopathy [1]
We focused on clinically relevant measures of efficacy and safety such as absolute change in forced vital capacity (FVC) % of predicted and diffusion lung capacity for CO (DLCO) % of predicted, number of patients with serious adverse events (SAEs), number of patients discontinuing treatment due to adverse events (AEs) and mortality
In this review and network meta-analysis (NMA) of nine randomized clinical trials (RCTs) investigating eight treatments and the placebo in a total of 926 patients with systemic sclerosis-related interstitial lung disease (SSc-ILD), only rituximab significantly reduced the decline of FVC % of predicted, but not of DLCO % of predicted, compared with the placebo
Summary
Systemic sclerosis (SSc) is an immune-mediated connective tissue disease (CTD) characterized by extensive fibrosis of the skin and internal organs and vasculopathy [1]. Pulmonary function tests (PFTs) typically demonstrate a restrictive pattern, with reduced forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO). There is a paucity of head-to-head comparisons of the efficacy and harms of pharmacological treatments for systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: We conducted a network meta-analysis (NMA) in order to compare the effects of different treatments with the placebo on change in forced vital capacity (FVC), change in diffusion lung capacity for CO (DLCO), serious adverse events (SAEs), discontinuation for adverse events and mortality in SSc-ILD. Only rituximab significantly reduced FVC decline (SMD (95% CI) = 1.00 (0.39 to 1.61)). Conclusion: Only rituximab significantly reduced lung function decline compared to the placebo. Direct head-to-head comparison studies are required to confirm these findings and to better determine the safety profile of various treatments
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