Abstract
Granulomatosis with polyangiitis is autoimmune and rare disease. It affects many organs, but the most often affected organs are the nose, lungs, and kidneys. It is part of vasculitis and causes an autoimmune attack by an abnormal type of circulating antibody termed ANCAs against small blood vessels. Disease concerns both men and women with a peak age of presentation in the sixth and seven decades. Typically upper and lower respiratory tract and kidneys are involved. Otitis externa, otitis media, or mastoiditis rarely occurs in granulomatosis with polyangiitis. Deafness is the most dangerous aural complication. Histological examination of biopsy is often not specific. A case of GPA with bilateral otitis media, bilateral deafness, and bilateral facial palsy with fatal course is presented.
Highlights
Granulomatosis with polyangiitis (GPA) is a rare, autoimmune, multisystemic disease first described by Friedrich Wegener in 1936 [1, 2] and has been named Wegener’s granulomatosis for many years
We present a rare case of progressive GPA with unusual presentation and fatal course
In patients with GPA, facial nerve palsy may develop in a classical form of a disease subsequent to middle ear involvement and occasionally in a locoregional form as primary otological manifestation accompanied by sensorineural hearing loss
Summary
Granulomatosis with polyangiitis (GPA) is a rare, autoimmune, multisystemic disease first described by Friedrich Wegener in 1936 [1, 2] and has been named Wegener’s granulomatosis for many years. GPA is necrotizing granulomatous inflammation involving the upper and lower respiratory tract and necrotizing vasculitis affecting predominantly small-to-medium vessels. GPA is a relatively rare condition, with a peak age of presentation in the sixth and seven decades of life. It can appear at any age, with no gender predilection [4]. Otologic symptoms may be present in the course of the disease but rarely are the first to appear [9,10,11]. We present a rare case of progressive GPA with unusual presentation and fatal course
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