Although primary Sjögren's syndrome is often a benign condition, characterized by lymphocytic infiltration of salivary and lacrimal glands, some patients develop systemic features.
We have previously found that anti-Ro antibodies identified patients with more systemic disease, with increased incidence of parotid swelling, lymphadenopathy and lymphoma.
Methods We have followed up a cohort of 100 patients over 10 yr, to establish whether the phenotypic expression of disease changed, and whether the different autoantibody patterns expressed at presentation could be used to predict outcome.
Result While seronegative patients (ANA, RF, Ro and La negative) remained polysymptomatic, they did not develop systemic complications or serological changes.
Thirty-nine per cent of ANA-or RF-positive patients who were negative for Ro and La were given revised diagnoses over the follow-up period, including rheumatoid arthritis, systemic lupus erythematosus. mixed connective tissue disease and scleroderma.
Parotid swelling and lymphadenopathy were more common in Ro/La-positive patients, where the relative risk of developing non-Hodgkin's lymphoma was 49.7. Conclusion.
Both HLA B8 and DR3 were present in 79% of Ro/La-positive patients, but were found together in only 4% of seronegative patients, supporting the view that these clinical subgroups of primary Sjögren's syndrome are both serologically and immunogenetically distinct. (...)
Mots-clés Pascal : Sjogren syndrome, Primaire, Anticorps, Autoanticorps, Phénotype, Système HLA, Epidémiologie, Angleterre, Grande Bretagne, Royaume Uni, Europe, Evolutivité, Lymphome, Tissu conjonctif pathologie, Pronostic, Homme, Oeil pathologie, Stomatologie, Maladie système, Immunopathologie, Maladie autoimmune, Hémopathie maligne, Lymphoprolifératif syndrome, Antigène Ro/SSA, Antigène La/SSB
Mots-clés Pascal anglais : Sjogren syndrome, Primary, Antibody, Autoantibody, Phenotype, HLA-System, Epidemiology, England, Great Britain, United Kingdom, Europe, Evolutivity, Lymphoma, Connective tissue disease, Prognosis, Human, Eye disease, Stomatology, Systemic disease, Immunopathology, Autoimmune disease, Malignant hemopathy, Lymphoproliferative syndrome
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0227841
Code Inist : 002B07. Création : 16/11/1999.