To determine the clinical and immunologic characteristics of a large cohort of patients with primary Sjörgen's syndrome (SS) and to asses if the sex, the age at onset, the time of evolution and the immunologic pattern define different subsets with specific characteristics.
We included 80 patients (76 female and 4 male) that were prospectively studied at our Unit. All patients fulfilled the European Community criteria proposed in 1993 for the diagnosis of SS.
Mean age of patients was 62 years with a mean disease duration of 8 years. The most frequently observed clinical manifestations were xerostomia (96%), xerophthalmia (94%) and parotidomegaly (46%). The main extraglandular manifestations were arthritis (45%), Raynaud's phenomenon (20%) and liver involvement (19%). The immunologic study showed antinuclear antibodies in 82% of patients, rheumatoid factor in 45%, anti-Ro/SS-A in 40% and anti-La/SS-B in 20%. In patients with an onset of disease before the age of 40 years, a higher prevalence of parotidomegaly, peripheral neuropathy, cutaneous vasculitis, rheumatoid factor, anti-Ro/SS-A and anti-La/SS-B antibodies was observed. A disease duration longer than 10 years was associated with a higher prevalence of pulmonary involvement and more focus of mononuclear cells in the minor salivary glands. Clinical manifestations associated to any one or more immunologic marker (rheumatoid factor, anti-Ro/SS-A and/or anti-La/SS-B) were Raynaud's phenomenon, arthritis, thyroid disease, cutaneous vasculitis and peripheral neuropathy.
Primary SS is an autoimmune disease characterized by a marked heterogeneity in the clinical presentation and evolution, thus allowing the definition of several subsets of patients with their own clinical and immunological characteristics.