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Abstract
Primary Sjögren's syndrome (SS) is a chronic inflammatory connective tissue disease, mainly affecting exocrine glands and leading to keratoconjunctivitis sicca (KCS) and xerostomia.
Anti-SS-B/La ab concentrations and prevalences of primary SS, defined as objectively verifiable KCS and xerostomia, were investigated in the general population (n = 705), primary biliary cirrhosis (PBC; n = 32), autoimmune thyroiditis (AT; n = 63), and mothers of offspring with isolated congenital complete heart block (CCHB; n = 5).
Among patients attending a rheumatology clinic, anti-SS-B/La ab, when tested with immunosorbent purified antigen in an enzyme-linked immunosorbent assay, had a sensitivity of 65%, a specificity of 91% and a predictive value of 78% for primary SS (at 33% prevalence).
Symptoms of dry eyes or mouth among 52-72-year-old individuals were reported by 247 (35%). Moderately increased anti-SS-B/La ab concentrations were found in symptomatic individuals and in some of the patients with PBC or AT, the concentrations being higher in symptomatic individuals than in controls (p < 0.001). The prevalence of primary SS in the general population was estimated to be 2.7% (1.0-4.5%).
Increased frequencies of autoimmune sialadenitis were found among cases of PBC (93%) and AT (21%), as compared to that of 2.7% in the general population. In PBC, autoimmune sialadenitis was distinguishable from that in primary SS by its significant IgM reaction. The prevalence of primary SS was ten times higher among patients with AT, and that of AT nine times higher among patients with primary SS, as compared with the general population.
Anti-SS-B/La ab at high concentrations, as well as abnormal objective testresults for KCS and xerostomia, were found in mothers of offspring with CCHB.