Treatment-recalcitrant laryngeal sarcoidosis responsive to sirolimus

BMJ Case Rep. 2020 Aug 26;13(8):e235372. doi: 10.1136/bcr-2020-235372.

Abstract

A 15-year-old girl presented with gradual-onset dysphonia and dysphagia. Laryngoscopy revealed significant supraglottic airway obstruction with swelling of both the epiglottis and arytenoids. After emergency tracheostomy, biopsy of the epiglottis revealed lymphoid hyperplasia with focal non-necrotising granulomata, leading to a presumed diagnosis of laryngeal sarcoidosis. Treatment with prednisolone and methotrexate produced minimal clinical improvement. A switch to sirolimus was followed by significant reduction in the laryngeal swelling, allowing decannulation of the tracheostomy. Treatment with sirolimus should be considered as a steroid sparing agent in laryngeal sarcoidosis, particularly in the presence of lymphoid hyperplasia on biopsy.

Keywords: otolaryngology / ENT; paediatrics; rheumatology.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Female
  • Humans
  • Laryngeal Diseases / drug therapy*
  • Sarcoidosis / drug therapy*
  • Sirolimus / therapeutic use*

Substances

  • Sirolimus