GRANULOMATOSIS WITH POLYANGIITIS WITH BILATERAL FACIAL PALSY AND ORAL ULCERS - AN ATYPICAL PRESENTATION

Granulomatosis with Polyangiitis (GPA) is a systemic vasculitis of autoimmune etiology, characterized by the triad: necrotizing granulomatous inflammation of the respiratory tract, disseminated vasculitis and glomerulonephritis. Early diagnosis requires a high level of suspicion, being essential the anatomopathological examination and the presence of antineutrophil cytoplasmic antibodies directed against proteinase 3 (c-ANCA). Among the ANCA - associated vasculitis, peripheral neuropathy is more commonly observed in eosinophilic granulomatosis with polyangiitis (60 – 80%) than in microscopic polyangiitis (40 – 50%) or GPA (20 – 25%). Neuropathy is not usually associated with life threatening manifestations, usually affects motor nerves and is long-lasting.