Abstract
Case presentation: A 10-year-old female patient with 6 months of stridor, non-productive cough and dyspnea on exertion. Repeatedly managed with systemic and nebulized corticosteroid. In the past 2 years, she manifested thick and crusty rhinorrhea, halitosis, epistaxis and 8 episodes of acute otitis media.
The study of stridor begins with a flexible nasolaryngoscopy with suspected laryngomalacia type 3 (posterior epiglotic colapse). She was taken to a rigid bronchoscopy, finding a grade II subglottic stenosis (airway obstruction between 51 and 70%). A neck CT scan showed decreased tracheal diameter related to subglottic stenosis (72% decreased diameter), and a chest CT scan reported disseminated lung nodules. Considering the clinical condition of the patient and the imaging findings, the diagnosis of granulomatosis with polyangiitis was considered. A positive anti-neutrophil cytoplasmic antibodies test by immunofluorescence: p-ANCA 1:320, and positive ELISA: anti-myeloperoxidase, confirmed the diagnosis. Immunosuppressive treatment was started with corticosteroids and cyclophosphamide monthly for 6 doses with an adequate clinical response, improvement of subglottic stenosis, and resolution of pulmonary nodules. Later maintenance was achieved with methotrexate.
Conclusion: Granulomatosis with polyangiitis is a rare disease in the pediatric population. High diagnostic suspicion and early treatment is required to improve the prognosis of patients.
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