Primary Sjogren's syndrome: clinical and immunological characteristics of 114 patients

BACKGROUND: To analyze the clinical and immunological characteristics of a series of 114 patients with primary Sjogren's syndrome (PSS), and to evalua te the different diagnostic criteria and the association to lymphoprolifera tive disorders. PATIENTS AND METHOD: We included 114 patients (108 female and 6 male) with a diagnosis of PSS. All patients fulfilled the 1993 European Community crit eria for the diagnosis of PSS and 76 patients fulfilled the San Diego Crite ria. RESULTS: Mean age was 51 years with a mean follow-up of 7.3 years. The comm onest clinical manifestation at onset (70%) was xerostomia/xerophtalmia (si cca syndrome). Extra glandular involvement was articular in 42% of cases, n eurologic (35%), respiratory (21%) and hepatic (13%), Eleven patients (9%) developed vasculitis, and three (2%) developed a lymphoproliferative disord er. No statistically significant differences regarding symptoms at onset, f requency of glandular or extra glandular manifestations and severity of dis ease were observed between the two diagnostic criteria groups. HCV infectio n was associated with vasculitis (p < 0.001; OR: 20.6; CI 95%, 3.2-129) and lymphoproliferative disorders (p < 0.001). CONCLUSIONS: The clinical evolution of PSS does not vary when using differe nt diagnostic criteria (San Diego and European Community criteria). A subse t of patients with vasculitis and lymphoproliferative diseases is found to have an associated HCV infection.