AB0943 CLINICAL AND LABORATORY PRESENTATION OF JUVENILE SJöGREN’S SYNDROME IN A COHORT OF 30 PATIENTS
Authors: Blanca Bica, Fernanda Zonis
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Sjögren's Syndrome (SS) is a chronic autoimmune disease, rare in childhood and adolescence, characterized mainly by xerostomia and xerophthalmia, which compromises exocrine glands causing inflammatory response that leads to glandular hyposecretion. SS is probably underdiagnosed in pediatric range due to differences in presentation regarding adults with this condition, causing low recognition of the disease in children. <h3>Objectives</h3> The authors describe demographic, clinical, laboratory and therapeutic profiles of a cohort of children with primary SS attended at a university center. <h3>Methods</h3> Retrospective analysis of 30 selected patients' medical records between 2005 and 2017 allowed collection of various data. Laboratory and additional investigations included documentation of ocular dryness (Schirmer test, Rose-Bengal stain); evidence of parotid involvement (scintiscan, sialometry); and histological evidence of lymphocytic infiltration of the minor salivary glands or other organs. <h3>Results</h3> 30 patients diagnosed with juvenile SS were selected: 22 girls (73%) and 8 boys (27%) with an average age of 11 years. The clinical characteristics were: parotid enlargement as the initial manifestation of the disease in 11 patients (37%), and recurrent episode in 4 patients (13,3%). 12 patients (40%) had xerostomia and 17 (57%) xerophthalmia. One patient (3,3%) presented with leukocytoclastic vasculitis in lower limbs as first manifestation of the disease in association to recurrent parotid swelling. Two (6,6%) patients presented neurological symptoms (1 peripheric sensory neuropathy and 1 with dysautonomic manifestations). Positive Schirmer test was observed in 11 patients (37%), Rose Bengal stain in 10 (33%). 76% of patients had abnormal salivary glands scintigraphy. In 8 patients (30%) the salivary gland biopsy revealed compatible with Sjogren syndrome. 9 patients (30%) presented positive RF, 14 patients (46%) anti-Ro/SSA, 10 patients (33%) anti-La/SSB, 28 patients (93%) had +ve ANA. 50% received glucocorticoid. Hydroxychloroquine was the drug most often used in 25 patients (83%), followed by methotrexate in 12 patients (40%), azathioprine in 4 patients (13%) and cyclophosphamide in 3 patients (10%). Only one patient required the use of human immunoglobulin and one leflunomide (3.3%). Two patients (6.6%) received rituximab. <h3>Conclusion</h3> The present study demonstrated the demographic, clinical aspects and laboratory and treatment in a series of patients with primary juvenile Sjögren's syndrome, a relatively rare condition, presenting an overview of this population in our hospital. <h3>References</h3> [1] Primary Sjogren syndrome in the paediatric age: a multicentre survey. Rolando Cimaz, Annachiara Casadei, Carlos Rose, et al. Eur J Pediatr (2003) 162: 661–665. [2] Primary Sjögren's syndrome in children and adolescents: Proposal for diagnostic criteria. J. Bartunková, A. Sedivá, J. Vencovsky, V. Tesar. Clinical and Experimental Rheumatology1999; 17: 381-386 [3] Primary Sjögren syndrome in childhood: Report of a case and review of the literature. Nikolaos G. Nikitakis, Helen Rivera, Carmela Lariccia, John C. Papadimitriou, and John J. Sauk. Oral Surgery Oral Medicine Oral Pathology Radiol Endod 2003;96:42-7. <h3>Disclosure of Interests</h3> None declared