Treatment-recalcitrant laryngeal sarcoidosis responsive to sirolimus

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.

Figure 1

Laryngoscopic appearance of supraglottis and vocal cords at (A) presentation, (B) after sequential treatment with methylprednisolone, oral prednisolone and methotrexate and (C) oral sirolimus. The severe supraglottic swelling seen in (A) had improved after initial treatment (B), but improvement was insufficient to allow decannulation of the tracheostomy. Complete resolution with normal epiglottic folds and vocal cords was seen (C) after sirolimus treatment.

Figure 2

CT images taken at presentation of airway obstruction from severe laryngeal sarcoidosis. Axial (A) and sagittal (B) view of CT of the neck demonstrating severe supraglottic laryngeal swelling leading to a pinhole airway (white arrows).

Figure 3

Histological appearance of epiglottic biopsy. Squamous mucosal tissue from epiglottic biopsy (magnified ×50) showing polypoid fragments, with extensive hyperplastic lymphoid infiltrates. Magnified side panels (×200, ×400 and ×200 from top) demonstrate numerous small discrete epithelioid granulomas (white arrows).