Mechanisms, clinical manifestations and management of cardiovascular diseases in ANCA-associated vasculitis

Chiara E, Bagni G, Raho A, Fagni F, Mohammad AJ, Emmi G (2026)

Publication Type: Journal article, Review article

Publication year: 2026

Journal

Rheumatology Oxford University Press

Book Volume: 65

Article Number: keag268

Journal Issue: 7

DOI: 10.1093/rheumatology/keag268

Abstract

ANCA-associated vasculitides (AAVs) are rare diseases characterized by small-vessel necrotizing vasculitis, multiorgan involvement and positivity for ANCAs. The main phenotypes are granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis, representing distinct yet partially overlapping entities in terms of pathogenesis, clinical expression and therapeutic management. Patients with AAV face a substantial cardiovascular (CV) and thrombotic risk, with higher rates of myocardial infarction, ischaemic stroke and venous thromboembolism than the general population. The excess CV burden reflects a complex, time-dependent interplay between disease-related inflammation, traditional CV risk factors and treatment-related toxicity, with inflammatory activity emerging as a key driver of early CV events. Across the disease course, this evolving risk profile requires multidisciplinary management to limit CV damage accrual and related mortality. This review integrates evidence on pathogenesis, clinical manifestations and management of CV disease in AAV, highlighting its time-dependent trajectory and key unmet needs.

Authors with CRIS profile

Filippo Fagni Department of Medicine 3 – Rheumatology and Immunology

Involved external institutions

Università degli Studi di Trieste IT Italy (IT) University of Turin / Università degli Studi di Torino (UNITO) IT Italy (IT) Lund University / Lunds universitet SE Sweden (SE)

How to cite

APA:

Chiara, E., Bagni, G., Raho, A., Fagni, F., Mohammad, A.J., & Emmi, G. (2026). Mechanisms, clinical manifestations and management of cardiovascular diseases in ANCA-associated vasculitis. Rheumatology, 65(7). https://doi.org/10.1093/rheumatology/keag268

MLA:

Chiara, Emanuele, et al. "Mechanisms, clinical manifestations and management of cardiovascular diseases in ANCA-associated vasculitis." Rheumatology 65.7 (2026).

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