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2015, Number 1

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Rev Cub de Reu 2015; 17 (1)

Juvenile primary Sjögren's Syndrome: Cohort study

Ríos GBBE, Saldarriaga RLM, de Almeida TH, Leitão AMN

Language: Spanish
References: 32
Page: 40-47
PDF size: 475.78 Kb.


ABSTRACT

Objective: To describe the demographic, clinical, and laboratory characteristics and management of patients with primary Sjögren 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 Sjögren 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 Sjögren's syndrome youth, a relatively rare condition, presenting an overview of this population in our hospital.


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