Santos De Sousa, Catarina Isabel2024-03-072024-03-072024Rev Port Cardiol. 2024 Mar;43(3):105-1060870-25512174-2030http://hdl.handle.net/10451/63235© 2023 Published by Elsevier España, S.L.U. on behalf of Sociedade Portuguesa de Cardiologia. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)Systemic vasculitides are rare autoimmune-mediated disorders affecting various organs. Their clinical presentation is usually heterogeneous, with a long and difficult period until a diagnosis is finally established. They are classified according to the type of affected vessels, pathogenesis, demographics and clinical characteristics. Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, is included in the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and is characterized by necrotizing vasculitis of small and medium-sized vessels. Ear, nose and throat, lungs, and kidneys are mainly affected, and the disease occurs more frequently in patients in the fifth decade of life.engjournal article10.1016/j.repc.2023.12.003