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 evaluate the different diagnostic criteria and the association to lymphoproliferative 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 criteria for the diagnosis of PSS and 76 patients fulfilled the San Diego Criteria. RESULTS: Mean age was 51 years with a mean follow-up of 7.3 years. The commonest clinical manifestation at onset (70%) was xerostomia/xerophtalmia (sicca syndrome). Extra glandular involvement was articular in 42% of cases, neurologic (35%), respiratory (21%) and hepatic (13%), Eleven patients (9%) developed vasculitis, and three (2%) developed a lymphoproliferative disorder. No statistically significant differences regarding symptoms at onset, frequency of glandular or extra glandular manifestations and severity of disease were observed between the two diagnostic criteria groups. HCV infection 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 different diagnostic criteria (San Diego and European Community criteria). A subset of patients with vasculitis and lymphoproliferative diseases is found to have an associated HCV infection.