Patients with different forms of
systemic vasculitis experience long-term morbidity and mortality caused by
cardiovascular disease due to premature
atherosclerosis. Epidemiologic reports of patients with GCA suggest that long-term mortality in this disease is not increased compared with the general population of the same age. The risk of a
stroke, however, in particular in the vertebrobasilar territory, is increased. In addition, the occurrence of aortic aneurysmal disease and
aortic dissection is also clearly increased in GCA. Mortality due to ischaemic
heart disease, however, is not increased. In
Takayasu arteritis accelerated
atherosclerosis has been clearly documented both clinically and in autopsy reports.
Atherosclerotic plaques in the carotid artery may be present in the carotid arteries especially in patients with a documented history of
arteritis involving the carotid artery. It is controversial whether
Kawasaki disease is associated with accelerated
atherosclerosis. Young adults with a history of
Kawasaki disease may have abnormal brachial artery reactivity, increased carotid IMT values and increased arterial stiffness. At autopsy examinations of KD patients, however, no significant atherosclerotic lesions are detected and carotid IMT measurements were found to be clearly different from those in young adults with familiar hypercholesterolaemia, suggesting that the remodeling process in KD is different from
atherosclerosis. In
ANCA-associated vasculitis (AAV), an increased mortality as a consequence of
cardiovascular disease is well-documented. In these patients the relative risk for
coronary heart disease is two- to fourfold that in control subjects. In addition, a similar relative risk has been found for
stroke. Diabetes,
hypertension,
dyslipidemia,
abdominal obesity (metabolic syndrome), impaired renal function, persistent
proteinuria and increased production of
C-reactive protein are common risk factors for premature
atherosclerosis in patients with
systemic vasculitis. Furthermore,
cholesterol and its modifications play a pivotal role in the pathogenesis of accelerated
atherosclerosis in
vasculitis. The (preventive)
therapy for accelerated
atherosclerosis in
systemic vasculitis is based on an aggressive approach against
inflammation and against risk factors of premature
atherosclerosis such as smoking, inactivity,
obesity and unhealthy diet. In addition, patients should be treated with
angiotensin-converting enzyme inhibitors and/or
angiotensin receptor-1 blockers for
hypertension and
statins for
dyslipidemia. Finally, low dose
acetylsalicylic acid should be prescribed in patients with large vessel
vasculitis, i.e., both in GCA and TA, who do not have
contraindications for ASA.