PReS-FINAL-2259: Spectrum of Sjögren syndrome in children

Results 26 patients were included in the study: 20 girls (76%), 6 boys (24%), with mean age at diagnosis of 12.6 years (3-21 years). Patients were 16 years of age or younger at the onset of symptoms. Eight (30.7%) patients had parotid gland enlargement as the initial manifestation of the disease, with recurrent episodes in 2 (7.6%) patients. Ten (38%) had dry mouth complaint and 16 (62%) had ocular signs and symptoms, 18 patients (69%) had altered parotid ultrasonography, 19 patients (73%) had impaired salivary glandscintigraphy and 6 patients (23%) had abnormal parotid MRI. In 8 patients (30%) the minor salivary gland biopsy showed chronic sialadenitis compatible with Sjögren syndrome. The analysis of serum autoantibodies, showed 8 patients (30%) positive for rheumatoid factor, 10 patients (38%) with Anti-Ro, 9 patients (34%) with Anti-La, 9 patients (34.6%) Anti-Ro and Anti-la and 18 patients (69%) with antinuclear antibody (ANA). 12 patients (46%) had arthritis. 7 patients (26%) had CNS involvement. 3 patients (11.5%) had vascular compromise. One patient (3.8%) showed tachyarrhythmia, leucopenia in 2 patients (7.6%), hypergammaglobulinemia in 8 patients (30%) and hypogammaglobulinemia in 1 patient (3.8%). With regard to treatment, 25 patients (96%) received hydroxychloroquine. Azathioprine was used in 4 patients (15.3%), methotrexate in 12 patients (46%), human immunoglobulin in 1 patient (3.8%), cyclophosphamide in 2 patients (7.6%), leflunomide in 1 patient (3.8%) and rituximab 2 patients (7.6%).