Abstract
to characterize the exocrine and non-exocrine clinical disease manifestations of 74 patients with primary Sjögren's Syndrome (pSS). retrospective study of pSS patients according to the new 2002 American-European criteria, followed in the Hospital Santa Maria, Rheumatology Outpatient Clinics, in the last 25 years (mean follow-up of 7.7 years). all patients presented surface exocrine disease, especially xerostomia and keratoconjunctivitis sicca. Internal organ exocrine disease was found in 25 patients (33.8%), whilst only one patient (1.4%) developed monoclonal B lymphocyte disease (pulmonary pseudolymphoma). Fifty-five patients (74.3%) also displayed non-exocrine manifestations, in particular inflammatory mediator-induced disease (68.9%). Inflammatory vascular disease was present in 54% of patients and noninflammatory vascular disease in 27%. Autoimmune thyroiditis was found in 5 patients (6.8%). the disease is dominated by oral and ocular involvement, while severe organic manifestations are less frequent. This classification model allows a comparison of patients with physiopathological abnormalities and offers the possibility of finding markers of activity/cronicity related to them.
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