Author(s)
Emma Marin Miller, MD
Scott Rickert, MD, FACS, FAAP
Affiliation(s)
Department of Otolaryngology-Head and Neck Surgery, NYU Grossman School of Medicine, New York, NY, USA
Abstract:
Introduction:
The average age for granulomatosis with polyangiitis (GPA) presentation is 45-60 years old with a female predominance. We present a case of clinical GPA in a young male presenting with gradual subglottic stenosis (SGS).
Case Description: A 17-year-old previously healthy male was referred for second opinion and management of presumed idiopathic SGS. Seven months prior to presentation, the patient developed recurrent spontaneous pneumothoraxes, chronic cough, and dyspnea. The patients’ prior evaluation included Pulmonary function tests (PFTs) consistent with a fixed upper airway obstruction, CT chests and neck demonstrating evolving subglottic and tracheal narrowing, direct laryngoscopies (DLs), rigid bronchoscopies notable for a nodular tracheal appearance; and extensive rheumatologic, oncologic, pulmonary, and infectious disease assessments. The patients’ prior laboratory work up was interpreted as inconclusive.
Initial biopsies were nondiagnostic; repeat bronchial biopsy demonstrated chronic inflammation with rare eosinophils consistent with asthma related changes. He was prescribed Flovent and Tezspire (tezelupumab-ekko) without improvement in his symptoms and briefly hospitalized in the intensive care unit (ICU) for acute hypoxic respiratory failure in the setting of a viral URI that improved with high dose steroids. An outside otolaryngologist performed a flexible fiberoptic laryngoscopy demonstrating SGS to 3-4mm. He was referred to a tertiary care children’s hospital for management.
The patient underwent repeat DL, rigid bronchoscopy with targeted biopsies, balloon dilation, and bronchoalveolar lavage. In the operative room, a saddle-nose deformity was appreciated and later confirmed as recently developed. High dose steroids were initiated and the patient was extubated on post operative day (POD) 1. Bronchial and bedside inferior turbinate biopsies demonstrated acute on chronic inflammation without evidence of granulomas or small vessel vasculitis. Repeat laboratory work up was notable for mildly elevated ANA and c-ANCA. The patient was discharged on POD 5 with a prednisone taper, bronchodilaters, and antibiotics. He has since initiated rituximab infusions for presumed GPA.
Discussion:
We present a case of GPA with negative biopsies, unremarkable laboratory findings, but classical physical exam findings consistent with GPA in a young male highlighting the importance of thorough otolaryngologic evaluation for diagnosis and management in this population.