Granulomatosis with polyangiitis (GPA) is a rare disease that belongs to the group of necrotizing systemic vasculitis, which is characterized by the involvement of small and medium-sized blood vessels, the formation of granulomas, and the presence of proteinase 3-anti-neutrophil cytoplasmic antibodies (PR3-ANCA). The disease typically involves the upper and lower respiratory tract and kidneys, but other organs and systems may also be involved. A rare manifestation of the disease is the involvement of large vessels, which may occur in the form of aneurysms, dissection and stenosis. We present the case of a 39-year-old male patient who was diagnosed with GPA several years ago, with the involvement of lungs, kidneys, skin and inflammation of the ascending aorta. The patient currently reported fever, cough and dyspnea. The imaging studies showed stenosis of the right and left pulmonary arteries. The treatment included glucocorticosteroids and rituximab. A review of the literature on pulmonary artery involvement in granulomatosis with polyangiitis was performed, seven case reports meeting such criteria were found.
