(091) Clinical Characteristics Among Pediatric Granulomatosis with Polyangiitis at Different Ages

Introduction: To evaluate and compare the specific clinical characteristics of pediatric granulomatosis with polyangiitis (GPA) involving ear-nose-throat (ENT) in different ages and provide basic references for clinicians regarding GPA diagnosis and treatment.

Methods: This study included 13 pediatric patients with GPA and ENT symptoms hospitalized at our hospital from April 2016 to July 2021. Participants were divided into two groups according to the age of immune development. Medical history, clinical features, original symptoms, concomitant symptoms and auxiliary examinations were evaluated and assessed in two age groups.

Results: Thirteen children were divided into younger group (≤7 years, 5 patients) and older group (＞7 years, 8 patients). Female preponderance was found in both groups (60% and 87.5% among younger and older GPA patients, respectively). ENT symptoms in pediatric patients were various, and the frequencies of all ENT signs were higher in older groups without significant difference (P＞0.05, respectively). The older groups had a higher rate of ocular signs (P=0.032). The two groups showed no differences in paranasal sinus imaging manifestations, while nodular lesion and cavitation in pulmonary CT significantly differed. No statistical significances were discovered in the level of PR3-ANCA between two groups. There was a significant difference in positive rate of biopsy between younger and older groups (P=0.05), which remains controversial due to the small sample research and diverse biopsy sites.

Conclusions: ENT symptoms are various and presented similar pattern among pediatric GPA in different ages. These older children experienced more ocular signs and nodules or cavity formation in the lungs. Patients present multiple ENT symptoms accompany with ocular lesions or pulmonary nodules should be suspected of having GPA, especially for older children.