RESUMO
Granulomatosis with polyangiitis is a systemic small-vessels vasculitis. It is characterized by a granulomatous inflammatory reaction around the arterial wall and is, in most cases, accompanied by the presence of cytoplasmic - type anti-neutrophil cytoplasmic antibodies (ANCA) with anti-proteinase 3 specificity. The predominant clinical presentation is frequent involvement of the upper and lower respiratory tract and the kidneys. This vasculitis is rarely revealed by pulmonary embolism. We report the observation of a 47-year-old male who presented with acute dyspnea. Chest X-ray disclosed an excavated opacity. Pulmonary angioscan revealed bilateral pulmonary embolism. The absence of clinical improvement after anticoagulant therapy with antibiotics and the presence of extra-respiratory signs led to the suspicion of a systemic inflammatory disease. The etiological investigations concluded with the diagnosis of granulomatosis with polyangiitis.