Sjögren’s syndrome in childhood

Abstract

This review presents our 10-year experience with children diagnosed with Sjögren's syndrome (SS). Patients between the ages of 9 and 17 years had abnormalities in laboratory values consistent with but not entirely diagnostic of those required to diagnose SS in adults. The spectrum of clinical manifestations suggests that the SS clinical phenotype in children is more variable than that in adults. Here, we review manifestations of SS in children. Our patients were treated with hydroxychloroquine, despite the lack of prospective data about effects on SS progression and/or autoantibody spreading. Patients have been followed for between 3 and 6 years without substantial progression of their disease or change in autoantibody status. Longer term follow-up (10-20 years) is needed to define the natural history of SS in childhood and its treatment outcomes. Prospective validation of SS criteria in childhood could facilitate assessment of the utility of hydroxychloroquine and other therapies.

Magnetic resonance imaging-based differentiation between juvenile recurrent parotitis and juvenile Sjögren’s syndrome

“…Salivary function is normal during periods of remission, but the salivary flow rate may be diminished during the active phase of the disease. These clinical and imaging features are very similar to those of juvenile Sjögren's syndrome (JSS), in which the symptoms and results of serological tests are more variable than those in the adult form [3]. Consequently, differentiation between these two distinct diseases on the basis of clinical and imaging examinations may be difficult, and even serological features and histological findings of the labial salivary gland may not be definitive.…”

A Boy With Sjogren’s Syndrome and Recurrent Parotitis Who Presented With Suspicious of Allergic and Immunological Disease: Review of the Literature

“…Primary SS is more common in adults, especially in middle-aged women, but it is rarely seen in children with female predominance and peak incidence at 10 years of age. It is a rare autoimmune disease at childhood, mainly affecting girls (77%) and those aged around 10 years of age [9]. In 1994, in a national survey from Japan, 61 cases recorded (nine in boys and 52 girls).…”

Primary Sjögren syndrome: report of a 10 years old girl with local edema and positivity of anti SS-A and anti SS-B autoantibodies

“…Symptomatic treatment for sicca symptoms, including artificial tears, salivary substitutes and systemic sialogogues, relieves the symptoms and prevents local infectious complications. Many studies showed that hydroxychloroquine may be useful in treating arthralgias, myalgias and asthenia in Sjögren patients (11,(17)(18)(19). Corticosteroids or other immunosuppressive agents should be reserved for cases showing evidence of organ damage, significant leukopenia or severe clinical manifestations (9,20).…”