Juvenile primary Sjogren's Syndrome: Cohort study

Objective: To describe the demographic, clinical, and laboratory characteristics and management of patients with primary Sjogren syndrome. Methods: Retrospective analysis of a cohort of 26 patients through review of medical records during the period February 2002 to December 2012. Results: 26 patients diagnosed with juvenile SS were selected: 20 girls (76 %) and 6 children (23 %) with an average of 12 years. The clinical characteristics were mumps as the initial manifestation of the disease in 8 patients (30.7 %), and recurrent episode in 2 patients (7.6 %). 10 patients (38 %) had xerostomia and 16 (61 %) xerophthalmia. The Schirmer test was altered in 9 patients (34 %), Rose Bengal in 10 (38 %). 73 % of patients had altered scintigraphy. In 8 patients (30 %) the salivary gland biopsy revealed Sjogren syndrome. 8 patients (30 %) FR-positive, 10 patients (38 %) anti-Ro/SSA, 9 patients (34 %) anti-La/SSB, 18 patients (69 %) ANA. 50% received glucocorticoid. Hydroxychloroquine was the drug most often used in 25 patients (96 %), followed by methotrexate and folic acid in 12 patients (46 %), azathioprine 4 patients (15.3 %) and cyclophosphamide in 2 patients (7.6 %). Only one patient required the use of human immunoglobulin and one leflunomide (3.8 %). Two patients (7.6 %) received rituximab. Conclusions: The present study demonstrated the demographic, clinical and therapeutic in a series of patients with primary Sjogren's syndrome youth, a relatively rare condition, presenting an overview of this population in our hospital.