Pediatric Vasculitis

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Diagnosis

Making the diagnosis of vasculitis is often challenging, as presenting symptoms may be subacute, nonspecific, and nondiagnostic. Fever, malaise, diffuse pain, and laboratory evidence of elevated acute-phase reactants may be the only early symptoms to suggest systemic inflammation. As vessel damage evolves, more specific clinical features, such as a purpuric rash, evidence of organ involvement, such as glomerulonephritis, or detection of certain antibodies, such as antineutrophil cytoplasmic

Classification

Primary vasculitis can be classified according to clinical manifestations, size of the affected vessels, or histopathology, including the presence or absence of granuloma. In 2005, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) developed the first pediatric-specific classification of vasculitis (Box 1).1 This classification system is primarily based on size of affected vessels and the presence or absence of granuloma.

Epidemiology and pathogenesis

The annual incidence of primary vasculitis in children and adolescents younger than 17 years is approximately 23 per 100,000.2 Primary vasculitis accounts for approximately 2% to 10% of all pediatric conditions evaluated in pediatric rheumatology clinics.3, 4, 5, 6 Of the primary vasculitides, Henoch Schönlein purpura (HSP) and Kawasaki disease (KD) are the most common, accounting for 49% and 23% of all childhood vasculitis, respectively.6 The prevalence of diseases may be different based on

Summary

Pediatric vasculitis is a challenging and complex group of conditions. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. The most common vasculitides are HSP and KD. Almost all knowledge about the optimal treatment and outcomes of children with vasculitis, with the exception of HSP and KD, have been adapted from adult studies or come from a small collection of case series. Early

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