Cardiac involvement and cardiovascular risk factors in pediatric primary systemic vasculitides

Clin Rheumatol. 2022 Nov 12. doi: 10.1007/s10067-022-06434-2. Online ahead of print.


Pediatric primary systemic vasculitides are a complex group of diseases. Vasculitis subgroups are mainly determined according to the size of the predominantly affected vessels. In patients with primary systemic vasculitis, the location of vascular involvement, the size of the vessels, the extent of vascular damage, and the underlying pathology determine the disease phenotype and severity. Cardiac involvement is rare in some pediatric vasculitis, such as IgA vasculitis and polyarteritis nodosa, while it is more common in some others like Kawasaki disease and Takayasu arteritis. On the other hand, chronic inflammation in the setting of systemic vasculitis forms a major cardiovascular risk factor. Accelerated atherosclerosis and the tendency to thrombosis are the main issues determining the cardiovascular risks in pediatric systemic vasculitis. Early diagnosis and treatment are essential in these patients to minimize morbidity and mortality. In this review, we aimed to raise physicians' awareness of cardiac involvement and cardiovascular risks in pediatric patients with primary systemic vasculitis.

Keywords: Behçet’s disease; Cardiac involvement; Cardiovascular risk factors; Kawasaki disease; Pediatric vasculitis; Takayasu arteritis.

Publication types

  • Review