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Vasculitis in children: Evaluation

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


Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely depending upon the type and location of the vessels involved and the extent of inflammation [1]. Because of the multisystem nature of these disorders, many pediatric subspecialties, including rheumatology, dermatology, nephrology, pulmonology, and cardiology, may be involved in the diagnostic workup and ongoing care of a child with vasculitis.

Although most types of primary vasculitis in childhood are rare, with an estimated incidence of 20.4 per 100,000 children below 17 years of age, prompt recognition and treatment of these disorders are important as these can be severe and life-threatening conditions without appropriate management.

The approach to evaluating a child with suspected vasculitis is reviewed here. The classification and overview of the management of childhood vasculitis are discussed separately. (See "Vasculitis in children: Classification and incidence" and "Vasculitis in children: Management overview".)


The diagnosis of primary vasculitis is challenging because the clinical presentation of childhood vasculitis varies widely depending upon the type and location of the vessels involved, the extent of inflammation, and subsequent vessel wall damage with associated hemodynamic changes (table 1) [1].

Many patients ultimately diagnosed with a specific vasculitis will have presented initially with a combination of fever or other constitutional symptoms, skin lesions, and laboratory evidence of inflammation. These early findings are neither specific nor sensitive for vasculitis and may be found in other conditions including common infections. In patients with such nonspecific systemic symptoms, vasculitis should be strongly considered if symptoms do not resolve as would be expected in a "self-limited" infectious illness. In addition to systemic symptoms, the presence of multiorgan involvement should heighten one's suspicion of vasculitis.

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Literature review current through: Oct 2017. | This topic last updated: Jan 27, 2016.
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