Background/Purpose: Sjögren syndrome in children and adolescents often presents differently from adult disease, with many pediatric patients failing to meet adult criteria. Additionally, treatment and outcomes in children and adolescents are not well defined. The objective of this study is to describe the current perspectives of pediatric rheumatologists on diagnosis, treatment and outcomes of pediatric Sjögren syndrome (pedSS).

Methods: A voluntary survey containing 17 questions was distributed via email to providers (pediatric rheumatologists, fellows, and advanced care providers) participating in the Childhood Arthritis and Rheumatology Research Alliance and/or the American College of Rheumatology Childhood Sjögren’s Study Group at the 2020 Annual Meeting. Descriptive statistics were used to report the findings.

Results: Between September 21 and November 16, 2020, 152 providers participated in the survey. The majority were pediatric rheumatology attendings from the United States. Most (85%) saw five or fewer, half (51%) saw one to two, and 15% had not seen any patients with pedSS in the past year. About three quarters (73%) felt pedSS is a spectrum of adult disease, while one quarter (24%) felt that pedSS is a distinct entity from adult disease. To make the diagnosis of pedSS, most (77%) use clinical judgment and/or experience guided by adult criteria. Parotitis, dry eye and/or dry mouth, and constitutional symptoms were among the most specific and most commonly observed symptoms. Arthralgias were more common but less specific, and dental caries were less common but more specific. Serologic tests were routinely used by >70% of providers for diagnosis. Amongst diagnostic tests, minor salivary gland biopsy was “always” used by 8% and “often” used by 43% of providers for diagnosis; other diagnostic tests were utilized less frequently. The most commonly prescribed systemic medications were hydroxychloroquine (85%), corticosteroids (67%), methotrexate (60%), rituximab (37%), and mycophenolate mofetil (34%). Providers perceived fatigue to be the most frequent symptom affecting quality of life, although only 15% would initiate systemic medication for fatigue. Providers also reported dryness and parotitis frequently affected quality of life for patients. Seven providers had observed malignancy in a patient with pedSS, including one death.

Conclusion: Pediatric rheumatologists are diagnosing and treating pedSS, although most providers see only a few patients per year. Most providers rely on clinical judgment and serologic testing for diagnosis, and some recognize pedSS as a distinct entity from adult disease. The most frequently prescribed systemic medications are hydroxychloroquine and corticosteroids, but substantial experience is reported with disease-modifying antirheumatic agents including methotrexate, mycophenolate mofetil, and biologics. Malignancy is observed in pediatric patients. More studies are critically needed to understand the natural history of pedSS, prognostic factors, and the impact of systemic medications on disease course and outcomes over time.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pediatric-rheumatologists-perspectives-on-diagnosis-treatment-and-outcomes-of-sjogren-syndrome-in-children-and-adolescents/