Abstract
To estimate the prevalence of Sjögren’s syndrome (SS) in the adult Spanish population we performed a population-based multicenter cross-sectional study. Cases were defined by the American-European Consensus Group criteria proposed in 2002. A total of 4,916 subjects aged 20 years or over were included. The estimated prevalence of SS (including primary and secondary forms) in the adult population in Spain was 0.33% (95% CI 0.21–0.53). Extrapolating to the total population of the country aged ≥ 20 years (around 37.7 million persons), there would be around 125,000 cases of SS in Spain. Considering only primary SS, the estimated prevalence was 0.25% (95% CI 0.15–0.43) or 1 person in 400. The prevalence of primary SS in Spain is comparable to that reported in other European studies with a similar design and diagnostic criteria. Based on these results, primary SS could not be considered a rare (orphan) disease. Only 50% of cases had already been diagnosed with SS prior EPISER 2016 study, confirming the existence of a non-negligible proportion of undiagnosed cases in the general population.
Highlights
To estimate the prevalence of Sjögren’s syndrome (SS) in the adult Spanish population we performed a population-based multicenter cross-sectional study
Using a cross-sectional population study, we found that the prevalence of Primary SS (pSS) in the general population of Spain was 0.25% or 1 person in 400
Even using the same classification criteria for pSS30, the prevalence rates in the studies that analyzed this issue are very much influenced by population size, case source, and study design
Summary
To estimate the prevalence of Sjögren’s syndrome (SS) in the adult Spanish population we performed a population-based multicenter cross-sectional study. The estimated prevalence of SS (including primary and secondary forms) in the adult population in Spain was 0.33% (95% CI 0.21–0.53). Extrapolating to the total population of the country aged ≥ 20 years (around 37.7 million persons), there would be around 125,000 cases of SS in Spain. The prevalence of primary SS in Spain is comparable to that reported in other European studies with a similar design and diagnostic criteria. Based on these results, primary SS could not be considered a rare (orphan) disease. It is critical to determine the prevalence of pSS to better understand its etiopathogenesis (assuming the existence of different rates between populations with different genetic and environmental backgrounds), and to determine the burden of the disease. In an era of increasing medical costs, financial constraints and managed
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