Background Sjögren syndrome (SS) is a chronic autoimmune disease characterized by a sicca syndrome and a wild spectrum of extra-glandular manifestations.

Objectives The aim of this study was to describe clinical, biological and immunological characteristics of patients with SS and to compare them in primary and associated SS.

Methods We conducted a monocentric, retrospective study over a period of 15 years. Patients who fulfilled the American European Consensus Group criteria for Sjögren syndrome were enrolled.

Results SS was diagnosed in 270 patients. The sex-ratio female/male was 10.73. The mean age at disease onset was 45 years +/- 13 years (range 15–74 years) and at diagnosis was 47 years +/- 13 years (range 15–76) with a mean delay of 3 years (range 0–25 years). Sicca syndrome revealed the disease in most cases; both ocular and buccal dryness (n=48), xerophtalmia (n=21), xerostomia (n=21) or parotid gland swelling (n=17). SS was also revealed by joint involvement (n=39), neurological manifestations (n=26) or interstitial lung disease (n=14). SS was systematically screened in 27 patients with another autoimmune disease and was found in 3 mothers who had a child with congenital atrio-ventricular bloc. Patients complained of xerophtalmia and xerostomia in respectively 95.2% and 95% of cases. Minor salivary biopsy was positive in 92.6% of cases. Parotid gland swelling was noted in 40 cases. Arthralgia and arthritis were respectively noted 77% and 18% of cases whereas myalgia and myositis were found in 11% and 5% of patients. Patients had pulmonary involvements in 27% of cases. Peripheral and central nervous system involvements were confirmed in 22% and 13% of cases and 12 patients presented with psychiatric disorders. Raynaud's phenomenon and purpura were noted in 48 and 17 patients. Biological data showed lymphopenia (n=115), anemia (n=83), thrombocytopenia (n=25) and hypergammaglobulinemia (n=151). Antinuclear antibodies were positive in 210 cases; anti-SSA and anti-SSB were present in respectively 57% and 37% of patients. SS was primary in 155 patients and was associated to another autoimmune disease in 113 patients; systemic lupus erythematosus (n=48), rheumatoid arthritis (n=20), systemic sclerosis (n=19), autoimmune liver disease (n=13) and auto-immune thyroiditis (n=9). Arthralgia (91.2% vs 66.2; p<0.0001), arthritis (35.7% vs 5.2%; p<0.0001), myalgia (17% vs 7.1%; p=0.012) and Raynaud's phenomenon (33.3% vs 7.7%; p≤0.0001) were significantly less frequent in primary SS. Lymphopenia (61.1% vs 32.9%; p<0.0001), anemia (48.6% vs 19.5%; p<0.0001), inflammatory syndrome (35.8% vs 17.9; p=0.002) and ANA (92% vs 70.9%; p<0.0001) were significantly more frequent in associated SS. Corticosteroids and immunosuppressive therapy were used in respectively 141 and 90 patients because of severe complications. Only 3 case of lymphoma were observed and 3 patients died. The mean duration of follow up was 50 months.

Conclusions Sicca syndrome is the major symptom of SS but extra-glandular manifestations are less frequent and can be serious causing disability specially neurologic and pulmonary involvements. Some manifestations are significantly more frequent in patients with associated autoimmune diseases like joint involvements, Raynaud's phenomenon, anemia and lymphopenia. These manifestations are mainly related to SLE, rheumatoid arthritis and systemic sclerosis.

Disclosure of Interest None declared