ANCA-negative associated vasculitis initially presenting with pulmonary embolism

Antineutrophil-cytoplasm antibodies (ANCA)-associated vasculitis are severe inflammatory pathologies that, although rare, may induce significant morbidity or death. Their impact on multiple organ systems implies an important variability in their clinical presentation, which might delay the diagnosis. In this setting we report on a case of ANCA-negative-associated vasculitis, initially presenting as pulmonary embolism with severe pulmonary infarction and digestive involvement. Literature is then discussed on these complications and their implications for therapy.