Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk

Abstract

Cardiac involvement is common in primary systemic vasculitides and may be due to direct effect of the disease on the heart or due to therapy. We shall review involvement of the heart in the various forms of primary systemic vasculitis. Among anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), eosinophilic granulomatosis with polyangiitis most commonly involves the heart. Involvement of the heart confers poorer prognosis in AAV, which is also complicated by increased risk of cardiovascular events. Kawasaki's disease (KD) is the most common form of medium-vessel vasculitis to affect the heart, with coronary artery aneurysms being the most common manifestation. These predispose patients with KD to develop premature ischemic heart disease. Takayasu's arteritis is the most common large-vessel vasculitis to involve the heart and can result in aortic incompetence, myocarditis, or coronary heart disease. Involvement of the heart in Behcet's disease is usually in the form of intracardiac mass lesions, thrombosis, or endomyocardial fibrosis. Drugs used in the treatment of systemic vasculitis influence the risk of developing cardiovascular events. Corticosteroid therapy has been shown to increase the risk of myocardial infarction, whereas methotrexate, azathioprine, mycophenolate mofetil, rituximab, and anti-tumor necrosis alpha agents favorably modulate the risk of cardiovascular events, predominantly by dampening systemic inflammation. Awareness of cardiac involvement in vasculitis and accelerated cardiovascular risk in these patients should help clinicians to maximize the modulation of modifiable risk factors for heart disease in these individuals.

Acute Myocarditis in Patients with Antineutrophil Cytoplasmic Antibody–positive Microscopic Polyangiitis and Receiving Rituximab Therapy

“…Nonspecific complaint in acute myocarditis such as dyspnea or chest pain is an underdiagnosed cause of acute heart failure 7 . Despite common presentation in admitted patients from this study, acute myocarditis has scarcely been reported in MPA 3 , raising the possibility of an overlooked diagnosis. The myocarditis-associated heart failure in MPA can have clinical symptoms and physical signs such as dyspnea and pedal edema mimicking the lung and renal involvement, respectively.…”

“…The presence of cardiac insufficiency is recognized as a poor prognostic factor in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) 1 , and heart failure is an identified mortality risk factor for eosinophilic granulomatosis with polyangiitis (EGPA) 2 . Microscopic polyangiitis (MPA) has lower frequencies of myocardial involvement with rarely reported acute myocarditis presentation 3 . Rituximab (RTX) is a first-line induction therapy licensed for severe MPA with reduced ANCA titers and clinical remission 4 .…”

Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

“…Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease occurring exclusively in asthmatic patients [1], and classi ed as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) [2]. Among 3 AAV-related disorders, cardiac involvement is most commonly observed in EGPA patients with a frequency ranging from 16 to 92% [3,4]. The therapy can be adapted for the presence of poor prognostic factors, i.e., ve-factor score (FFS) [5].…”

FRI0351 Papulopustular lesions according to age, sex and localization in behcet's syndrome patients compared healthy and diseased controls

“…Subclinical endothelial injury as a result of chronic inflammation in BD has been associated with thrombosis and arterial stiffness, risk factors for ischemic heart disease (IHD) 1-4 . However, data regarding the association between BD and IHD and the impact of various risk factors on this association, is scarce [3][4][5] .…”