CASE HISTORY – September 2010

Wegener’s granulomatosis in the upper airways: a case report and a review of the literature


Articles PDF


A 61-year-old female presented with a onemonth history of otalgia, otorrhea and high fever (39 ºC). She also complained of nasal obstruction and left-sided hearing loss. Examinat ion revealed left-sided otitis media with pus effusion. Anterior rhinoscopy revealed diffuse swelling of the nasal mucosa. She had failed to respond to systemic antibiotics used earlier. A myringotomy was performed. Pure tone audiometry demonstrated mixed hearing loss in the left ear. A biopsy was taken from the nasal mucosa. Histologically, all criteria for the diagnosis of WG were fulf illed: inflammatory infiltration and necrotizing changes as well as presence of giant cells in vessel walls. Serum autoantibodies against neutrophils (c-ANCA test) appeared to be positive. The patient was treated with systemic prednisolon and antibiotics. Also the left ear was regularly cleaned.

For further investigation and treatment, the patient was sent to the department of internal medicine.  Wegener’s granulomatosis (WG) is vasculitis of small and medium sized vessels typified by necrotizing inflammation of the upper airways, the lower respiratory tract and the kidneys. The etiology of the disease is still unclear. WG usually starts as a limited and localized organ manifestation in the upper respiratory tract and, if untreated, generalizes with pulmonary and renal involvement. Initial presentation of WG may involve only head and neck symptoms, which are observed in up to 92% of the patients. These include recurrent rhinosinusitis, nasal congestion, crusting, epistaxis, nose deformity, dyspnea, and alterations of voice, as well as symptoms of otitis media, mastoiditis ans sensorineural hearing losss. Untreated WG usually has a progressive fatal course. Therefore, early diagnosis of WG is of utmost importance since immunosupressive therapy is associated with significantly improved life expectancy. The otorhinolaryngologist plays an important role in the early diagnosis and follow-up owing to frequent manifestation of the disease in the upper airways.