Cardiac involvement in small and medium-sized vessel vasculitides

Abstract

The heart can be involved in vasculitides but the frequency of its involvement and the manifestations vary according to the vasculitis. Cardiovascular manifestations include cardiomyopathy (specific or resulting from myocardial infarctions), coronary arteritis (with risk of aneurysms, stenoses and thrombosis formation or rupture), pericarditis, valvulitis, conduction-tissue involvement (with heart blocks), arrhythmias (mainly supraventricular) and/or dissection of the aorta (and/or its proximal branches). As many of these manifestations are clinically silent, at least during their early stages, heart function should be systematically assessed in vasculitis patients, with at least ECG and echocardiography, and more invasive exploratory procedures when the former reveal abnormalities or symptoms become manifest. Specific cardiomyopathy has been identified as a factor of poor outcome in small and medium-sized vessel vasculitides (five-factor score). Therefore, in addition to symptomatic treatments, prescription of corticosteroids and immunosuppressants (mainly cyclophosphamide) is considered mandatory. This regimen has dramatically improved the overall prognosis of affected patients.