Background/Purpose: Sjögren syndrome (SS) is a chronic autoimmune disease of exocrine gland dysfunction that affects up to 4 million adults in the United States. Children are much less commonly affected, but have been reported to present with distinct, sometimes severe, manifestations. Little is known about clinical features, treatment and outcomes of SS in children.

Methods: An institutional web-based clinical research tool was used to identify patients with Sjögren or sicca syndrome diagnosis codes from year 2000-current in any child (< 18 years old at time of diagnosis). Query revealed 237 charts which were manually reviewed and narrowed to 25 cases of SS diagnosed by a pediatric rheumatologist, for whom in-depth chart review was performed.

Results: Of all 25 cases, 19 (76%) had primary SS and 6 (24%) had SS in the context of another autoimmune disease. Of those with primary SS, 10 (53%) had recurrent parotitis and 6 (32%) had sicca syndrome as predominant presenting symptom; 2 (10%) developed severe, persistent central nervous system symptoms. Of those with other autoimmune disease, 3 (50%) had a diagnosis of autoimmune disease prior to onset of SS, and 3 (50%) were initially diagnosed with SS and subsequently developed other autoimmune disease (undifferentiated connective tissue disease or systemic lupus erythematosus). Frequently reported symptoms included arthralgia, headache and fatigue. Nearly all (96%) had high ANA and SSA/Ro antibody titers. Shirmer test and salivary gland biopsy were less commonly used (44% and 36%, respectively). The most frequently prescribed systemic treatments were hydroxychloroquine, methotrexate, prednisone and methylprednisolone. Long-term outcome data were not available for the majority of cases, however there were no reports of malignancy or death, and only one case where the disease was not controllable with systemic medications.

Conclusion: In this single center study of 25 cases of SS in children, clinical presentation was variable and included recurrent parotitis, sicca syndrome and severe neurologic symptoms, both with and without other autoimmune disease. Although follow up data were not available for the majority of cases, it seemed that disease was controlled in nearly all cases using systemic therapies during treatment course. Long-term studies are needed to better understand the natural history and outcomes of SS in children.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pediatric-sjogren-syndrome-a-single-center-experience/