Granulomatosis with polyangiitis (GPA) is a rare systemic disease of unknown aetiology, characterised by necrotising granuloma formation and diffuse vasculitis. It typically affects the upper and lower respiratory tract in addition to the kidneys. Without treatment, the 2-year mortality rate exceeds 90%. We describe a presentation of intractable serous otitis media, rhinosinusitis, fever and unilateral facial palsy in an 18-year-old man with learning difficulties, which highlights the need for a high index of suspicion in diagnosing and treating this enigmatic disease.
- nose and throat/otolaryngology
- respiratory medicine
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Contributors KYG and RCD reviewed the patient when he first attended clinic, treated the patient, collected data and drafted and revised the case report. RB reviewed the patient and drafted and revised the case report. HND obtained patient consent and revised the case report. BR helped in conceptualising, preparing, editing and approving this case report besides helping in the management of the patient. RCD is the clinical supervisor overseeing care of the patient and conceptualised, drafted, edited and revised the case report.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.