title: Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients.
abstract: We conducted the current study to characterize the clinical presentation of primary Sjögren 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 937 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.
authors.affiliation: From Laboratory of Autoimmune Diseases "Josep Font," Department of Autoimmune Diseases, IDIBAPS, Hospital Clínic (MRC), Barcelona; Department of Internal Medicine, Hospital Vall d'Hebron (RS), Barcelona; Rheumatology Unit, Hospital de Villajoyosa (JR), Alicante; Department of Internal Medicine, Hospital Carlos Haya (MTC), Malaga; Department of Internal Medicine, Hospital La Paz (AG), Madrid; Department of Internal Medicine and Rheumatology, Hospital Universitario de Salamanca (JdPM), Salamanca; Rheumatology Section, Hospital de Sierrallana (JCA), Torrelavega; Department of Internal Medicine, Hospital Virgen de las Nieves (JJA), Granada; Department of Internal Medicine, Hospital La Fe (MLM), Valencia; Rheumatology Unit, Hospital General de Castellón (JB), Castellón; Rheumatology Unit, Hospital 9 d'Octubre (RB), Valencia; Department of Internal Medicine, Hospital de Son Dureta (LP), Palma de Mallorca; Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Roser
authors.initials: R
authors.lastname: Solans
authors.forename: Jose
authors.initials: J
authors.lastname: Rosas
authors.forename: María Teresa
authors.initials: MT
authors.lastname: Camps
authors.forename: Antonio
authors.initials: A
authors.lastname: Gil
authors.forename: Javier
authors.initials: J
authors.lastname: Del Pino-Montes
authors.forename: Jaime
authors.initials: J
authors.lastname: Calvo-Alen
authors.forename: Juan
authors.initials: J
authors.lastname: Jiménez-Alonso
authors.forename: Maria-Luisa
authors.initials: ML
authors.lastname: Micó
authors.forename: Juan
authors.initials: J
authors.lastname: Beltrán
authors.forename: Rafael
authors.initials: R
authors.lastname: Belenguer
authors.forename: Lucio
authors.initials: L
authors.lastname: Pallarés
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000032:analysis; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D012859:Sjogren's Syndrome / Q000453:epidemiology / Q000276:immunology* / Q000503:physiopathology; D013030:Spain / Q000453:epidemiology
chemical_list: D015415:Biomarkers