Primary Sjögren's syndrome: clinical spectrum and mode of presentation based on an analysis of 50 patients selected from a department of rheumatology.

In this study we report on the clinical characteristics at the time of referral and during follow-up of 50 patients with primary Sjögren's syndrome (SS) in a rheumatological practice. The mean age of the patients at the time of diagnosis was 51.5 years. Four of them were men and 46 women. During a mean follow-up period of 9.1 yr (1-21 yr) from symptom onset the most common signs and symptoms were arthralgia (94%), complaints of dryness of the eyes (90%), mouth (82%) and skin (56%), weariness (64%), exanthema (48%), Raynaud's phenomenon (42%) and arthritis (42%). An elevated erythrocyte sedimentation rate (72%), elevated serum gammaglobulin (49%) and leucocytopenia (42%) were the most frequently encountered abnormalities of routine laboratory tests. After the initial hospital evaluation the diagnosis primary SS was made in only 10 (20%) of the patients. The diagnostic delay was on average 3.1 yr. Knowledge of the considerable heterogeneity of the disease may prevent extensive evaluations and provide better clinical care for patients with primary SS.