Case of paediatric relapsing polychondritis with severe airway involvement: the challenges of long-term airway and respiratory management

Abstract

We describe a case of a 15-year-old girl diagnosed with relapsing polychondritis (RP) with involvement of the tracheobronchial tree, resulting in an increased difficulty in breathing, hoarseness of voice and stridor.Her case required the input of multiple specialities including ear, nose and throat, rheumatology, respiratory team and intensive care. Airway assessment and imaging showed glottic and subglottic stenosis and left bronchomalacia. Despite the use of steroids, cyclophosphamide and rituximab, her symptoms progressed and she was started on overnight non-invasive ventilation.She had further relapses of her airway RP-she was started on adalimumab and methotrexate and underwent monthly balloon dilatations which helped with her symptoms and facilitated a safe discharge home. However, she had further relapses and intensive care admissions and following further discussions, a tracheostomy was inserted. She is now stable on her tracheostomy and is off any respiratory support.

Keywords: ear; nose and throat/otolaryngology; paediatrics; rheumatology.