Jornal de Síndromes Genéticas e Terapia Gênica
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Cardiovascular Magnetic Resonance for Evaluation of Heart Involvement in ANCA-Associated Vasculitis: A Luxury or a Valuable Diagnostic Tool?

Mavrogeni S and Kolovou G

Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg–Strauss syndrome (CSS). ANCA-associated vasculitis usually presents with lifethreatening kidney failure or pulmonary hemorrhage, has a mortality rate of 28% at 5 years, and contributes to increased morbidity and morbidity.

Cardiac involvement in this entity includes coronary vessels vasculitis, pericarditis, myocarditis, endocarditis, myocardial infarction and diffuse subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can reveal noninvasively and without radiation, early heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients.