Improving Diagnosis of Childhood Sjögren Disease via a Multidisciplinary Clinic

Introduction: We have previously reported a surprisingly high rate of Childhood Sjogren’s Disease (cSD) patients who present with recurrent parotitis. For this reason, we created a joint rheumatology-otolaryngology clinic. The purpose of this study is to review the outcomes of this unique clinic.

Methods: Retrospective analysis of patients seen in this multidisciplinary clinic between December 2019 and July 2022 presenting with recurrent parotitis was performed. Demographic information, clinical symptoms, and lab/biopsy/imaging results were collected. All patients were diagnosed with cSD by a pediatric rheumatologist based on evidence of recurrent sialadenitis, focal lymphocytic infiltrate on biopsy, abnormal serologies, family history, and/or systemic symptoms.

Results: Twenty-seven children presented to the clinic; fourteen (51.9%) patients were diagnosed with cSD. In comparison, eight children were diagnosed with cSD over a 10 year period prior to implementation of this clinic. Seven (50%) of the patients with cSD were female with a median age of symptom onset of 6.5 (1-15) years. In addition to all having recurrent parotitis, 4 (28.6%) had joint pain, 9 (64.3%) had dry eye symptoms, 10 (71.4%) had dry mouth symptoms, and 2 (14.3%) had recurrent idiopathic fevers. All underwent a lower lip biopsy and/or parotid biopsy, and 11 underwent sialendoscopy. Thirteen (92.9%) showed focal lymphocytic infiltration on biopsy. Five (35.7%) had anti-SSA, anti-SSB and/or ANA autoantibody positivity.

Conclusions: A multidisciplinary joint rheumatology and otolaryngology clinic appears to improve the detection and diagnosis of cSD in children who present with recurrent parotitis.