Abstract
INTRODUCTION: Despite significantly improved therapies in recent years, long-term morbidity and mortality in ANCA-associated vasculitis (AAV) remain high. The leading causes of death within the first year after diagnosis are active vasculitis and in subsequent years cardiovascular diseases, malignancies, and infections. Population-based database and cohort analyses suggest an increased risk for major adverse cardiovascular events (MACE) in AAV. METHODS: This retrospective cohort study analyzed data samples from an electronic health records database of the US-based TriNetX network. Patients with the diagnostic codes granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and patients without vasculitis as a matched control cohort (1:1) were included. To optimize between-group comparability, propensity score matching was performed for demographic variables and comorbidity. Hazard ratios (HR) for death and cardiovascular outcomes were calculated using univariate Cox regression after analyzing the matched cohort using the Kaplan-Meier method. RESULTS: We identified 20, 422 patients with GPA and 5, 907 with MPA. Mortality was more frequent in patients with GPA (17.87%) and MPA (25.85%) than in matched controls (GPA controls: 5.79%; MPA controls: 9.70%), corresponding to an increased hazard of death in both cohorts (GPA: HR 3.01; MPA: HR 3.01). The risk of cardiovascular events was increased in GPA and MPA compared to matched controls, particularly for MACE (GPA: HR: 1.94, MPA: HR: 2.24) and thromboembolic events (deep vein thrombosis: GPA HR: 2.82, MPA HR: 3.33; pulmonary embolism: GPA HR: 3.01, MPA HR: 3.00) and did not differ when adjusted according to sex, disease duration, and age. Compared with GPA patients, MPA patients had a higher risk of MACE (HR: 1.13) and peripheral arterial disease (HR: 1.17). CONCLUSION: AAV was associated with an increased risk of death and cardiovascular events. Compared with GPA, MPA was associated with an increased risk for MACE and peripheral arterial disease.