Assessment of the mortality burden of systemic sclerosis in the Russian Federation: results of a systematic review and meta-regression analysis

Objective: to develop a population-based mortality model for patients with systemic sclerosis (SSc) and to assess the disease burden (mortality indicators) for the Russian Federation.

Material and methods. A systematic review was conducted in the PubMed and Embase databases. Eleven real-world clinical practice studies were selected. Data analysis was performed in R v.4.3.0 using a random-effects meta-analysis model and meta-regression analysis. Covariates included the proportion of patients with diffuse disease and the proportion of patients with interstitial lung disease. For forecasting indicators for the Russian Federation, the proportions of patients were assumed to be 40% and 60%, respectively. Risk of bias was assessed using the ROBINSIv2 tool.

Results and discussion. Based on bivariate meta-regression analysis, SSc mortality in Russia was estimated at 26.1 deaths per 1000 patientyears (95% confidence interval, CI 17.3–39.3). The standardized mortality ratio was 4.2 (95% CI 2.6–6.5), indicating a 4.2-fold higher risk of death compared with the general population. The obtained estimates are consistent with international data and confirm the determining role of diffuse disease and lung involvement in shaping mortality.

Conclusion. SSc in Russia is associated with a high risk of premature mortality. The developed meta-regression model provides valid preliminary estimates of disease burden in the absence of a national registry. The results emphasize the need for early detection of lung involvement, standardized screening, and establishment of a national registry to monitor disease course and treatment effectiveness. 

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