PATIENTS with anti-neutrophil cytoplasmic antibody-associated vasculitides face substantially higher risks of cardiovascular disease and thromboembolism than the general population, a nationwide matched cohort study has found.
Nationwide Register-Based Cohort Design
In this nationwide matched cohort study, adults diagnosed with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) were identified from national patient registers between 2005 and 2020, with five age- and sex-matched population controls randomly selected per patient, alongside first-degree siblings of patients and controls.
Outcomes, defined using ICD codes, included myocardial infarction, ischemic or haemorrhagic stroke, deep vein thrombosis, pulmonary embolism, cardiovascular or thromboembolism-related death, and a composite endpoint. Hazard ratios with 95% confidence intervals were estimated using Cox proportional hazards models.
Disease Subtype and Sex-Specific Risk Patterns
The study included 4,317 patients with vasculitis, 21,582 controls, and 25,568 siblings. Both GPA and MPA were associated with increased composite cardiovascular and thromboembolic risk compared with controls (HR 2.04, 95% CI 1.83 to 2.38, and HR 2.45, 95% CI 2.00 to 2.99), with no significant differences between subtypes. Both sexes showed increased deep vein thrombosis and pulmonary embolism risk. In GPA, only males had increased myocardial infarction risk (HR 2.25, 95% CI 1.74 to 2.92), while only females had increased ischemic stroke risk (HR 1.64, 95% CI 1.25 to 2.17). Siblings showed no excess risk. Risk peaked within three months of diagnosis (HR 7.69, 95% CI 5.93 to 9.99).
Implications for Early Cardiovascular Monitoring
These findings have established comparable, substantially elevated cardiovascular and thromboembolic risk across GPA and MPA, concentrated most heavily in the first three months after diagnosis, with distinct sex-specific patterns in GPA. The absence of excess risk among siblings suggests the vasculitis itself, rather than shared familial or genetic susceptibility, drives this risk, supporting close cardiovascular monitoring immediately following diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitides.
Reference
Lindberg H et al. Cardiovascular and thromboembolic risk in anti-neutrophil cytoplasmic antibody-associated vasculitis: a nationwide matched cohort study by disease, sex, and familial risk. Rheumatology. 2026;keag467.
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