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Is primary Sjögren's syndrome an orphan disease? A critical appraisal of prevalence studies in Europe
  1. Divi Cornec1,
  2. Laurent Chiche2
  1. 1Service de Rhumatologie, CHRU Brest, ESPRI/ERI29, Université de Bretagne Occidentale, Brest, France
  2. 2Service de Médecine Interne, Hôpital de la Conception, AP-HM, Université Aix-Marseille, Marseille, France
  1. Correspondence to Dr Divi Cornec, Service de Rhumatologie, Hôpital de la Cavale Blanche, BP 824, Brest, Cedex F 29609, France; divi.cornec{at}

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A meta-analysis of epidemiological studies in primary Sjögren's syndrome (pSS) has been recently published in the Annals of the Rheumatic Diseases by Qin et al.1 According to this study, the estimated prevalence of pSS worldwide is 60.82 per 100 000 inhabitants, or 1 person in 1644. Thus, the prevalence of pSS would not fit in with the definition of a rare disease. However, the extraordinary heterogeneity of the results between the different included studies is striking: the prevalence of this disease in single studies ranges from 11.34 to 3790.09 per 100 000 persons.

In Europe, a disease is considered rare when it affects less than one person per 2000: this is the definition of an orphan disease. To date, 6000 to 7000 rare diseases have been recognised, but this figure is constantly evolving with scientific knowledge ( Many are genetic diseases, some are infectious or autoimmune. The recognition of a disease as rare has important implications, most importantly, for the development of new therapies. Indeed, …

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