Maladie de Sjögren : rare ou fréquent ?

While several criteria for classification have been used for epidemiological studies about primary Sjögren syndrome (pSS), it remains unclear if pSS is an orphan disease. Among 11 analyses retrieved from 9 population-based studies, 8 show a prevalence lower than the orphan disease threshold (50/100,000 inhabitants). In two meta-analyses, prevalence is 39 to 43 per 100,000 inhabitants. There is no difference among prevalence in studies using or not classification criteria. ACR/EULAR 2016 criteria have not been used yet. Women are 6 to 9-fold more involved than men in pSS, and incidence for diagnosis is at the highest between 60 and 70 years old. Although Asian people are the most likely to present pSS, Whites are also more likely than Blacks or Hispanics. Collectively, pSS shall be now considered as an orphan disease, with important implications for clinicians, for development of new therapies and for national health programs.