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Revista chilena de pediatría

Print version ISSN 0370-4106

Abstract

LADINO R, Mabel; GASITULLI O, Angelina  and  CAMPOS M, Ximena. Sjögren's syndrome: case report. Rev. chil. pediatr. [online]. 2015, vol.86, n.1, pp.47-51. ISSN 0370-4106.  http://dx.doi.org/10.1016/j.rchipe.2015.04.009.

Introduction: Sjögren’s syndrome (SS) is a chronic autoimmune disease that primarily affects the lacrimal and salivary exocrine glands. In children, it is a rare condition. Objective: To present the case of an adolescent with non-specific symptoms, but with a clinical suspicion of SS. Case report: A male 12-year old patient, with history of arthralgias for 3 years and suspicion of xerophthalmia. Physical examination showed mild conjunctival congestion, dry mouth and hypermobility of the knees. Laboratory work: blood count and ESR were normal, antinuclear antibodies (+) > 60, Ro (+) > 60 U, and rheumatoid factor concentration (+) 160 IU/ml. SS was suspected, and a study was carried out: Schirmer test determined mild dry eye, salivary gland scintigraphy showed parotid and submandibular gland dysfunction, and salivary gland biopsy reported focal lymphocytic acinar and periductal infiltration. SS was confirmed and treated with prednisone 7.5 mg/day and hydroxychloroquine 200 mg/day, and local treatment, with good response. Conclusions: The diagnostic criteria for SS in adults identified only 39% of pediatric patients, due to the low frequency of sicca symptoms. Still there are no validated diagnostic criteria for children. A good diagnosis will alleviate symptoms, prevent complications and detect associated diseases.

Keywords : Sjögren Syndrome; Pediatrics; Diagnosis; Keratoconjunctivitis sicca.

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