Abstract
Abstract
Background
Childhood Sjögren’s Disease (cSjD) is an underdiagnosed phenomenon with clinical and pathophysiological nuances in contrast to Sjögren’s Disease (SjD) in the adult population. While adults typically experience sicca symptoms, children with cSjD often present with recurrent parotitis, diverse autoantibody profiles, and renal and neurological manifestations. Diagnosis and classification in pediatric rheumatology remain controversial due to the reliance on adult-focused diagnostic criteria and the lack of standardized treatment and understanding of outcomes. The purpose of the paper is to propose a multimodal treatment plan and demonstrate the effectiveness of sialendoscopy in the management of cSjD.
Case Presentation
We present the case of a twelve-year-old female diagnosed with cSjD using the 2016 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) diagnostic criteria for SjD. In addition to medical management, she underwent sialendoscopy with triamcinolone irrigation under sedation and was monitored for progress via salivary gland ultrasonography (SGUS). Over the course of one year, she demonstrated significant improvement in symptoms, with serial SGUS scores gradually decreasing by five points.
Conclusions
This paper proposes a multimodal treatment plan involving sialendoscopy and medical management as a non-invasive and potentially more effective approach for cSjD. Standardized monitoring through SGUS scoring allows objective and quantifiable measurement of treatment progress, enabling better assessment of glandular tissue status. Recurrence is possible, and each cSjD patient may present differently. Nevertheless, our year-long observation of a patient with cSjD demonstrates that sialendoscopy, as seen in adults, can promote remission of recurrent parotitis in children as well.
Publisher
Springer Science and Business Media LLC
Subject
Immunology and Allergy,Rheumatology,Pediatrics, Perinatology and Child Health
Reference22 articles.
1. Randell RL, Stern SM, Van Mater H, et al. Pediatric rheumatologists’ perspectives on diagnosis, treatment, and outcomes of Sjögren disease in children and adolescents. Pediatr Rheumatol Online J Sep. 2022;05(1):79.
2. Yokogawa N, Lieberman SM, Sherry DD, Vivino FB. Features of childhood Sjögren’s syndrome in comparison to adult Sjögren’s syndrome: considerations in establishing child-specific diagnostic criteria. Clin Exp Rheumatol 2016 Mar-Apr 2016;34(2):343–351.
3. Vivino FB. Sjogren’s syndrome: clinical aspects. Clin Immunol 09. 2017;182:48–54.
4. Takagi Y, Sasaki M, Eida S, et al. Comparison of salivary gland MRI and ultrasonography findings among patients with Sjögren’s syndrome over a wide age range. Rheumatol (Oxford) May. 2022;05(5):1986–96.
5. Basiaga ML, Stern SM, Mehta JJ, et al. Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria. Rheumatology (Oxford). 2021;07(7):01.
Cited by
1 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献