Granulomatosis with polyangiitis (Wegener's; GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which also includes microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). GPA is characterized by granulomatous inflammation and necrotizing vasculitis predominantly affecting small- to medium-sized blood vessels and the presence of ANCA directed to specific antigens, particularly proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA). The destructive inflammatory processes of GPA have a predilection for the upper and lower airways and the kidneys, but any organs can be affected. An increased incidence of various cardiovascular events has been demonstrated among GPA patients. Cardiac involvement is also an independent predictor of mortality in GPA patients. In this prospective cohort study, consecutive GPA patients who are hospitalized in the Department of Family Medicine, Internal and Metabolic Diseases at the Medical University of Warsaw in Poland are included. All patients are diagnosed with GPA according to current guidelines. Patients are entered into the study at the time point when a new diagnosis of GPA was established and received initial treatment at our centre. Data collection included a full interim medical history, physical examination, laboratory studies and review of adverse events. Additionally, in all patients an echocardiography is performed.
Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. Inflammation-induced thrombosis is considered to be a feature of systemic autoimmune diseases. GPA usually involves the upper and lower respiratory tract and renal systems, where necrotizing glomerulonephritis and pulmonary capillaritis are often detected. However, it may also affect other organ systems. Cardiac involvement in GPA occurs in approximately 6% to 44% of cases and is secondary to necrotizing vasculitis with granulomatous infiltrates. Cardiac involvement is an independent predictor of mortality in GPA patients. In this prospective cohort study, consecutive GPA patients who were hospitalized in the Department of Family Medicine, Internal and Metabolic Diseases at the Medical University of Warsaw in Poland are included. In all patients echocardiography and laboratory tests are perform.