Epidemiology of primary Sjögren's syndrome in a French multiracial/multiethnic area.

Abstract

OBJECTIVE:

To describe the epidemiology of primary Sjögren's syndrome (SS) in a multiracial/multiethnic population.

METHODS:

A cross-sectional study with 5 case-retrieval sources identified adults with primary SS living in the Greater Paris area (population 1,172,482 adults) in 2007. Diagnoses were verified by the American-European Consensus Group (AECG) criteria and study-specific enlarged criteria based on the presence of ≥3 of 4 AECG items among subjective oral or ocular dryness, anti-SSA/SSB positivity, and positive minor salivary gland biopsy results. Prevalence estimates were standardized to those for the world population and a 5-source capture-recapture analysis (CRA) was used. Racial/ethnic differences in primary SS features were evaluated.

RESULTS:

In all, 133 subjects met the AECG criteria and 203 met the enlarged criteria. The 2007 prevalence of primary SS was 1.02 cases per 10,000 adults (95% confidence interval [95% CI] 0.85-1.22) for the AECG criteria and 1.52 cases per 10,000 adults (95% CI 1.30-1.76) for the enlarged criteria. The CRA indicated completeness of case findings of ∼90%. Compared to subjects with European backgrounds, those with non-European backgrounds had 2.1-2.3 times higher primary SS prevalence and were younger (P < 0.0001) and were more likely to have polyclonal hypergammaglobulinemia (P < 0.0001) and anti-SSA/SSB antibodies (P = 0.0005 and P < 0.0001 for the AECG and enlarged criteria, respectively).

CONCLUSION:

The figure of 1.02–1.52 cases per 10,000 adults we found and estimates from the few other population-based census surveys support that the prevalence of diagnosed primary SS is between 1 and 9 cases per 10,000 (0.01-0.09%) [corrected] in the general population. Non-European race/ethnicity may be associated with increased primary SS risk and a distinct disease profile.

• Authors: Carla Maldini, Raphaèle Seror, Olivier Fain, Robin Dhote, Zahir Amoura, Michel De Bandt, Jean-Luc Delassus, Géraldine Falgarone, Loïc Guillevin, Véronique Le Guern, François Lhote, Olivier Meyer, Jacky Ramanoelina, Karim Sacré, Yurdagul Uzunhan, Jean-Louis Leroux, Xavier Mariette, Alfred Mahr
• Journal: Arthritis care & research (Arthritis Care Res (Hoboken)) Vol. 66 Issue 3 Pg. 454-63 (Mar 2014) ISSN: 2151-4658 [Electronic] United States
• PMID: 23983119 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 by the American College of Rheumatology.

Topics

• Adult
• Aged
• Cross-Sectional Studies
• Female
• France (epidemiology)
• Humans
• Male
• Middle Aged
• Prevalence
• Retrospective Studies
• Sjogren's Syndrome (ethnology)