Abstract Background Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA) – associated vasculitis. The disease is characterized by necrotising inflammation of small vessels causing tissue ischemia in a variety of organs with a wide range of clinical presentations. It has been established that active GPA is associated with a very high risk of venous thromboembolic events (VTEs). Purpose The aim of the present study was an evaluation of inflammation, coagulation and fibrinolysis markers, and their association with various clinical and laboratory parameters in GPA patients. Methods The group of 100 consecutive patients with GPA was prospectively followed in the study. In all patients echocardiography and laboratory tests were performed. Results The patients were followed up for an average of 4.0±1.9 years. In this group, D-dimer was elevated in majority (56%) of patients. The circulating levels of D-dimer were significantly higher in GPA patients in active stage of disease compared with those in remission (652 vs. 405 ng/ml, p=0.0002). In 23 patients venous thromboembolism was diagnosed during observation. However, there was no difference in D-dimer concentration between patients with and without VTE either in active stage or in remission. Among the 100 GPA patients higher D-dimer levels were observed in patients with coronary artery disease (p=0.04), atherosclerosis (p=0.01), hypertension (p=0.0049), diabetes (p=0.0065) and hypercholesterolemia (p=0.03). Moreover, correlation analysis showed that the levels of D-dimer correlated with hs-CRP (r=0.42; p<0.0001) and creatinine (r=0.58; p<0.0001). In patients with VTE, D-dimer levels correlated positively with age (r=0.5, p=0.02), right ventricle diameter (r=0.63, p=0.0017), right ventricle systolic pressure (r=0.44, p=0.04), and negatively with pulmonary artery acceleration time (r=−0.43, p=0.05). D-dimer – normal D-dimer – elevated p Inclusion – active stage n=44 (44%) n=56 (56%) Elevated creatinine 1 (2.27%) 25 (44.6%) <0.0001 Elevated CRP 5 (11.3%) 22 (39.2%) 0.0015 Venous thromboembolism 7 (15.9%) 16 (28.6%) 0.0712 CRP: C-reactive proteine. Conclusion In patients with Granulomatosis with polyangiitis elevated levels of D-dimer are associated with disease activity and inflammation, rather than with risk of venous thromboembolism. D-dimer is not a useful marker of venous thromboembolism episodes in patients with small vessel vasculitis.