Vasculitis Syndromes

Vidya Sivaraman, Edward C. Fels, Stacy P. Ardoin

Childhood vasculitis encompasses a broad spectrum of diseases that share

inflammation of the blood vessels as the central pathophysiology. The

pathogenesis of the vasculitides is generally idiopathic. Some forms of vasculitis

are associated with infectious agents and medications, whereas others may occur

in the setting of preexisting autoimmune disease. The pattern of vessel injury

provides insight into the form of vasculitis and serves as a framework to

delineate the different vasculitic syndromes. The distribution of vascular injury

includes small vessels (capillaries, arterioles, and postcapillary venules), medium

vessels (renal arteries, mesenteric vasculature, and coronary arteries), and large

vessels (the aorta and its proximal branches) (Fig. 192.1 ). Additionally, some

forms of small vessel vasculitis are characterized by the presence of

antineutrophil cytoplasmic antibodies (ANCAs) , whereas others are

associated with immune complex deposition in affected tissues. A combination

of clinical features, histologic appearance of involved vessels, and laboratory

data is used to classify vasculitis (Tables 192.1 to 192.3 ). A nomenclature

system from the 2012 International Chapel Hill Consensus Conference has

proposed using the pathologic diagnosis rather than eponyms for vasculitis

nomenclature. For example, Henoch-Schönlein purpura would be referred to as

IgA vasculitis. Additionally, the classification criteria endorsed by the European

League Against Rheumatism, Pediatric Rheumatology International Trial

Organization, and Pediatric Rheumatology European Society (EULAR/

PRINTO/PRES) have been validated in childhood vasculitis. (Table 192.1 ).