[Systemic signs of primary Gougerot-Sjögren syndrome. 48 cases]

Forty-eight patients with primary Sjögren syndrome are documented together with the results of their baseline investigations. The majority of patients were female (44 out of 48) and mean age was 63.2 years. Common clinical features included 20 parenchymal lung disease, among which 4 had interstitial pulmonary fibrosis and 2 lymphocytic interstitial pneumonitis, 12 neurologic manifestations, 16 Raynaud's phenomenon, 12 arthritis, and 3 gastrointestinal involvement. Haematological features occurred in 15 patients and another autoimmune disease was encountered in 13 cases. These extraglandular manifestations were the dominating reasons for hospital referral in 43.5% of cases, the sicca syndrome were most often only mentioned by the patients after special questioning which explain considerable delay before the diagnosis.