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Background: In spite of significantly improved therapy during the last years, long-term morbidity and mortality has remained high in ANCA-associated vasculitis (AAV). The main causes of death within the first year after diagnosis are active vasculitis and infections, followed by cardiovascular disease, malignancies and infections during subsequent years 1-3. Population-based database and cohort analyses suggest an increased risk for major adverse cardiovascular events (MACE) in AAV 4, 5. Objectives: To assess risk of death and cardiovascular outcomes in AAV using a large global electronic health record database for biomedical and clinic research (TriNetX) 6. Methods: In this retrospective cohort study, data samples from an electronic health records database of the US-based TriNetX network were analysed. Patients (aged≥18 years) with the diagnostic codes of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) (International Classification of Diseases ICD) and patients without vasculitis as matched control cohort (1: 1) were included. Propensity score matching for demographic variables and cardiovascular and metabolic comorbidities was performed to optimize between-group comparability. Hazard ratios (HR) related to death and cardiovascular outcomes (MACE) were calculated by univariate Cox regression after analysis of the matched cohort by the Kaplan-Meier method. P values were determined by the Log-rank test. Results: We identified 21. 190 patients with GPA and 5. 907 with MPA. The unadjusted and adjusted relative risk (RR) of mortality was increased in GPA (RR 17. 87%) and MPA (RR 25. 85%) compared to matched controls (GPA: HR: 3. 010 P P Figure 1A). The risks of all screened cardiovascular events were increased in GPA and MPA compared to matched controls (P Figure 1A) and did not differ when adjusted according to gender, disease duration (first 3 years after diagnosis), and age (patients 64 years). Especially the risk for thromboembolic events was increased compared to matched controls (deep vein thrombosis: GPA HR: 2. 819, MPA HR: 3. 334; pulmonary embolism: GPA HR: 3. 009, MPA HR: 2. 997). Compared to GPA, MPA patients had a higher risk for MACE (MPA vs. GPA: 24. 38% vs. 23. 76%, HR: 1. 160 P = 0. 0023) and peripheral arterial disease (MPA vs. GPA: 21. 38% vs. 21. 17%, HR: 1. 165 P = 0. 0018; Figure 1B). Conclusion: In AAV, the risk of death and cardiovascular events were increased. Compared to GPA, MPA was associated with an increased risk for MACE and peripheral arterial disease. REFERENCES: 1 Lamprecht P et al. Pathogenetic and clinical aspect of anti-neutrophil cytoplasmic autoantibody-associated vasculitides. Front Immunol. 2018 Apr 9;9: 680. 2 Sanchez-Alamo B et al. Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2023;38₁655-1665. 3 Flossmann O et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70: 488-494. 4 Aviña-Zubieta JA et al. Risk of Myocardial infarction and stroke in patients with granulomatosis with polyangiitis (Wegener's): A population-based study. Arthritis Rheumatol. 2016 Nov;68 (11): 2752-2759. 5 Massicotte-Azarnioch D et al. Association of anti-neutrophil cytoplasmic antibody-associated vasculitis and cardiovascular events: a population-based cohort study. Clin Kidney J. 2021 Nov 24;15 (4): 681-692. 6 Palchuk et al. A gobal federated real-world data and analytics platform for research. JAMIA Open 2023;6 (2). Acknowledgements: NIL. Disclosure of Interests: None declared.
Klapa et al. (Sat,) studied this question.