Identifier to cite or link to this item: http://hdl.handle.net/20.500.13003/13867
Primary Sjogren syndrome in Spain - Clinical and immunologic expression in 1010 patients
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ISSN: 0025-7974
eISSN: 1536-5964
WOS ID: 000257894100004
Scopus EID: 2-s2.0-49149117769
PMID: 18626304
Embase PUI: L354617105
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2008-07Document type
research articleCitation
Ramos-Casals Manuel, Solans Roser, Rosas Jose, Teresa Camps Maria, Gil Antonio, Del Pino-Montes Javier, et al. Primary Sjogren syndrome in Spain - Clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008 Jul;87(4):210-219.Abstract
We conducted the current study to characterize the clinical presentation of primary Sjogren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. The cohort included 93 7 women and 73 men (ratio, 13: 1), with a mean age of 53 years at diagnosis and 59 years at inclusion in the registry. Multivariate analysis showed that male patients had a lower frequency of thyroiditis, Raynaud phenomenon, and antinuclear antibodies. Young-onset patients had a low degree of sicca involvement (xerostomia and parotid enlargement) and a high frequency of immunologic markers (anti-Ro/SS-A and low C4 levels). Patients with disease duration of more than 10 years had a higher prevalence of xerophthalmia, parotid enlargement, lung involvement, and peripheral neuropathy in comparison with incident cases. The subset of patients with anti-Ro/La antibodies had the highest prevalence of most systemic, hematologic, and immunologic alterations (higher frequency of Raynaud phenomenon, altered parotid scintigraphy, positive salivary gland biopsy, peripheral neuropathy, thrombocytopenia, and rheumatoid factor). Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia. Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement.
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https://dx.doi.org/10.1097/MD.0b013e318181e6afMeSH
Aged, 80 and overAged
Age Factors
Spain
Adult
Humans
Adolescent
Middle Aged
Male
Biomarkers
Sex Factors
Female
Cohort Studies
DeCS
Estudios de CohortesBiomarcadores
Femenino
Adolescente
Masculino
Factores Sexuales
Humanos
Persona de Mediana Edad
Factores de Edad
Anciano
Anciano de 80 o más Años
Adulto
España