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Vasculitis in children: Classification and incidence

David Cabral, MBBS, FRCPC
Kimberly Morishita, MD, MHSc, FRCPC
Section Editor
Robert Sundel, MD
Deputy Editor
Elizabeth TePas, MD, MS


Vasculitides are disorders defined by the presence of inflammation in a blood vessel wall (vasculitis). The inflammation may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely depending upon the types and location of the vessels involved, the extent of inflammation, and subsequent vessel wall damage with associated hemodynamic changes.

The primary vasculitides may be classified according to their clinical manifestations, the size and type of the involved blood vessels, and the pathologic changes found within the vessel walls [1].

The nomenclature and criteria used for classification of the primary childhood vasculitides will be reviewed here. The approach to the child with suspected vasculitis and an overview of the management of children with vasculitic disorders are presented separately. The classification and approach to vasculitides in adults are also discussed elsewhere. (See "Vasculitis in children: Evaluation" and "Vasculitis in children: Management overview" and "Overview of and approach to the vasculitides in adults".)


Vasculitis is rare in children as demonstrated by the following two population-based studies:

In an English survey of family clinicians, the estimated overall annual incidence of new cases of vasculitis was 53.3 per 100,000 children under 17 years of age [2]. The two most common vasculitides were immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura) and Kawasaki disease, with estimated annual incidences of 20.4 and 5.5 per 100,000 in children less than 17 years of age, respectively.

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Literature review current through: Oct 2017. | This topic last updated: Mar 25, 2016.
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