ANCA-associated refractory vasculitis with multiple systemic involvement: A rare case report

We report the case of a 65 year old female patient, presenting with a combination of bilateral hearing loss, otalgia, and hyperacusis. Pure tone audiometry revealed mixed bilateral hearing loss. Conventional cranial imaging tests failed to show a significant brain pathology, but fat-suppressed T1-weighted gadolinium-enhanced magnetic resonance imaging scan displayed a diffuse infiltrative skull base process, extending from the nasopharynx to the jugular fossa, and encasing the internal carotid artery. The latter findings, besides elevated inflammatory markers and a positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) led to the diagnosis of ANCA-associated vasculitis. Additional disease manifestations sequentially appeared, including a right peripheral nerve palsy, aortitis, hepatitis, peripheral neuropathy, and uveitis. Therapy with corticosteroids, azathioprine, and then cyclophosphamide brought no evident benefit, but rituximab led to impressive clinical and radiologic improvement.