Granulomatosis with Polyangiitis (GPA)—A Multidisciplinary Approach of a Case Report

Abstract

Granulomatosis with polyangiitis is an atypical, multisystem disease with unknown etiology that generally affects both genders equally, with a predominance in the Caucasian racial group for individuals in their fourth decade. The disease affects the small vessels of the respiratory system, lungs, and kidneys. ENT manifestations are common, but ocular involvement is also frequent and can occur as an initial harbinger of the disease. The signs and symptoms of the disease are non-pathognomonic and sometimes localized, but it carries a poor prognosis if left untreated. Early diagnosis of granulomatosis with polyangiitis can be difficult and is established by a clinical examination along with laboratory tests for anti-neutrophil cytoplasmic antibodies (ANCA) and anatomopathological exam results that showcase necrosis, granulomatous inflammation, and vasculitis. Although the ocular involvement is not life threatening, it can cause blindness and may also be a sign of the active form of this systemic fatal disease. Treatment strategies involving immunosuppression and adjuvant therapies improve the prognosis. In this article we present a rare case of a patient diagnosed with granulomatosis with polyangiitis in our ENT department in 2003, with a follow-up for19 years in our clinic.

Keywords

• otorhinolaryngology
• granulomatosis with polyangiitis
• interdisciplinary
• vasculitis
• ocular manifestations
• diagnosis