Year: 2013
Type: article
Study design: Observational Study
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.
Source: Arthritis Care & Research
DOI: 10.1002/acr.22115
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
Institutions: Hôpital Saint-Louis, Université Paris Diderot, Université Paris-Sud, Inserm, Hôpitaux universitaires Paris Sud, Université Sorbonne Paris Nord, Hôpital Jean-Verdier, Hôpital Avicenne, Pitié-Salpêtrière Hospital, Université Pierre-et-Marie-Curie, Centre hospitalier Robert-Ballanger, Hôpital Cochin, Université Paris Descartes, Centre Hospitalier Saint-Denis, Hôpital Bichat-Claude-Bernard, Groupe Hospitalier Intercommunal Le Raincy Montfermeil
Language: English
Subfield: Physiology (medical)
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W1925295969