What is the prevalence and other epidemiologic features of Sjögren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Answer

In the United States, Sjögren syndrome is estimated to be the second most common rheumatologic disorder, behind SLE. Sjögren syndrome affects 0.1-4% of the population. This wide range, in part, reflects the lack of uniform diagnostic criteria. [26] Internationally, comparative studies between different ethnic groups have suggested that Sjögren syndrome is a homogeneous disease that occurs worldwide with similar prevalence and affects 1-2 million people.

The female-to-male ratio of Sjögren syndrome is 9:1. Sjögren syndrome can affect individuals of any age but is most common in elderly people. Onset typically occurs in the fourth to fifth decade of life.

The systemic phenotype of primary Sjögren syndrome is strongly influenced by personal factors (eg, age, gender, ethnicity, place of residence, according to an analysis by the Sjögren Big Data Consortium, a five-continent multicenter registry, of a cohort that included 10,007 patients (9352 female, mean age 53 years) with recorded European League Against Rheumatism's Sjögren syndrome disease activity index (ESSDAI) scores available. [27] Findings (all P < 0.001) were as follows:

  • Males had a higher mean ESSDAI than females (8.1 vs 6.0, respectively).
  • Patients diagnosed at < 35 years had a higher mean ESSDAI than those diagnosed at > 65 years (6.7 vs 5.6).
  • By ethnicity, the highest global ESSDAI scores were reported in blacks/African Americans (6.7), followed by whites (6.5), Asians (5.4), and Hispanics (4.8).
  • Black/African American patients showed the highest frequencies in the lymphadenopathy, articular, peripheral and central nervous system, and biological domains.
  • White patients showed the highest frequencies in the glandular, cutaneous, and muscular domains.
  • Asian patients showed the highest frequencies in the pulmonary, renal, and hematological domains.
  • Hispanic patients showed the highest frequenies in the constitutional domain.
  • Systemic activity and disease activity was higher in patients from southern countries.

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