Idiopathic Laryngeal Granulomatosis in a teenage girl responsive to Sirolimus

Background: Laryngeal granulomatosis may occur in isolation or in conjunction with systemic diseases including ANCA-associated vasculitis and Sarcoidosis. There are a number of case reports of Idiopathic Laryngeal Granulomatosis occurring in teenagers, with little evidence as to what might constitute effective treatment modalities. Case: A previously well 13 year old girl presented with non-infectious necrotising & suppurative granulomatous cervical lymphadenopathy and went on to develop upper airway obstruction, with reduced exercise tolerance and obstructive sleep apnoea requiring CPAP. Laryngoscopy revealed swelling of the supraglottis and epiglottis, with biopsy showing granulomatous inflammation. No underlying diagnosis could be identified. She was relatively non-responsive to systemic or intraepiglottic steroids, debulking procedures, topical laser therapy or immunosuppression with mycophenolate (MMF) or Adalimumab. Sirolimus therapy led to a marked reduction in her supraglottic oedema and resolution of her symptoms including sleep apnoea. Conclusion: Sirolimus was extremely effective in treating protracted refractory Idiopathic Laryngeal granulomatosis, with reduced airway swelling and symptom resolution.