Myeloperoxidase Positive Wegener’s Granulomatosis with Isolated Otorhinological

Abstract

Abstract:Introduction: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract and kidney. Case Report: We present here the uncommon case of a 71year old Caucasian man with newly diagnosed GPA presenting with isolated otorhinological involvement. The patient described 3 weeks of right-sided otalgia, mastoid pain and right hemifacial pain with associated rhinorrhoea, particularly at night, fevers and sweats. On examination, the right mastoid was tender to palpation but neither erythematous nor swollen. A Computed Tomography (CT) brain scan showed right-sided sinusitis and mastoiditis and right-sided acute on chronic otitis media without bony erosion. A biopsy of the right maxillary sinus found suppurative granulomatous changes, mild active vasculitis with neutrophil polymorphs in vessel walls and healing vasculitis with sclerosed vessels in keeping with a diagnosis GPA. Serology showed that he was p-anti-neutrophil cytoplasmic antibody (p-ANCA) positive, myeloperoxidase (MPO) positive and serine proteinase 3 antigen (PR3) negative. His GPA was treated with prednisolone in the acute phase and azathioprine was later commenced for maintenance therapy. Conclusion: This case highlights the need to consider GPA in a patient presenting with isolated otorhinological involvement without organ involvement.