Rare Epiglottic Granulomatosis: Diagnostic Challenges and Therapeutic Implications.

Abstract

We report a rare case of epiglottic granulomatosis with polyangiitis (GPA) in a 72-year-old woman presenting with 5 years of progressive dysphagia and dyspnea. Initial laboratory studies revealed leukocytosis (White Blood Cells 14.2 × 10³/µL), eosinophilia (8%), elevated inflammatory markers (C-Reactive Protein 42 mg/L, Erythrocyte Sedimentation Rate 78 mm/hour), and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody (PR3-ANCA) positivity (65 IU/mL). Laryngoscopy identified supraglottic edema with a right-sided mass, confirmed by Computed Tomography as localized epiglottic thickening without cartilage destruction. Histopathology of the biopsied lesion demonstrated necrotizing granulomas, vasculitis, and multinucleated giant cells, establishing the GPA diagnosis. Treatment with high-dose corticosteroids (1 mg/kg/day prednisone) and rituximab (375 mg/m²/week × 4) induced rapid symptom resolution within 2 weeks. Follow-up laryngoscopy at 1 month showed complete mucosal healing, and the patient remains recurrence-free during 18 month surveillance with maintenance immunosuppression (5 mg/day prednisone). This case highlights 3 critical insights: (1) isolated epiglottic GPA, although rare, should be considered in chronic upper airway obstruction unresponsive to conventional therapy; (2) PR3-ANCA serology and histopathology are indispensable for diagnosis; and (3) rituximab-based regimens achieve excellent outcomes in laryngeal GPA, but prolonged monitoring is essential given relapse risks. Our findings reinforce the need for multidisciplinary management of otolaryngologic GPA manifestations.