Sjögren's Syndrome in Children

Abstract

Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the lacrimal and salivary glands; it eventually leads to keratoconjunctivitis sicca and xerostomia. The disease usually affects middle-aged women and is second to rheumatoid arthritis as the most commonly diagnosed connective tissue disorder. Surprisingly, only 31 cases have been reported in the pediatric population. The diagnosis should be considered, however, in children with a foreign body sensation in the eyes, chronic dryness of the eyes or mouth, or recurrent salivary gland enlargement. This has prompted us to describe our experience in treating five children with SS at Texas Children's Hospital, Houston. One 5-year-old child with no other evidence of connective tissue disease was diagnosed as having primary SS. The remaining four children either had juvenile rheumatoid arthritis or systemic lupus erythematosus in addition to SS, and the diagnosis of secondary SS was made. Other autoimmune disorders associated with SS include polyarteritis nodosa, scleroderma, and polymyositis. Interestingly, one child with secondary SS and chronic lymphocytic thyroiditis developed papillary carcinoma of the thyroid. Both forms of SS are probably more prevalent than the literature would suggest. Recognition of this fact is important if the immediate complications of corneal damage and severe dental decay are to be prevented. Symptomatic treatment with steroids, eyedrops, artificial saliva, and prophylactic fluoride treatments is available. With an adequate follow-up program the late morbidity associated with SS, such as malignant disease, can be minimized.