Granulomatosis with Polyangiitis Manifesting as Refractory Otitis Media and Mastoiditis

Abstract

Granulomatosis with polyangiitis (GPA) is a rare rheumatologic disease characterized by small to medium vessel vasculitis and inflammation within multiple organ systems. Majority of cases involve both upper and lower respiratory tracts but other organs including brain, kidneys, joints and skin can also be involved. Patients who have recurrent otitis media and sinusitis often initially present to internists and then to ear nose and throat (ENT) physicians and may be treated with multiple courses of antibiotics without having proper workup for GPA. We present a middle-aged white male who exemplifies this unique presentation with new onset recurrent otitis media and mastoiditis, which did not respond to repeated courses of antibiotics requiring mastoidectomy and myringotomy tube placement. On chest x-ray, he was found to have multiple lung nodules that was followed by a computed tomography (CT) scan of his chest and CT guided biopsy which revealed granulomatous inflammation and necrosis, consistent with GPA. Autoimmune laboratory work-up was also suggestive of GPA. The patient had complete resolution of symptoms with steroid therapy. This case reinforces the concept of high index of suspicion of GPA for patients with recurrent and resistant otitis media or mastoiditis not responding to optimal duration and doses of antibiotics. Appropriate work-up for GPA may allow for earlier diagnosis of this devastating vasculitic disease which can help prevent multi-organ dysfunction.

Keywords: ANCA associated vasculitis, Granulomatosis with polyangiitis, Otitis media, Recurrent mastoiditis