Pediatric Vasculitis
Section snippets
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
References (59)
- et al.
Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins
Lancet
(2002) - et al.
Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schönlein purpura
J Pediatr
(2003) - et al.
Henoch Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature
Semin Arthritis Rheum
(2005) Kawasaki syndrome: a 1986 perspective
Rheum Dis Clin North Am
(1987)- et al.
Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial
J Pediatr
(2003) - et al.
A predictive instrument for coronary artery aneurysms in Kawasaki disease. US Multicenter Kawasaki Disease Study Group
Am J Cardiol
(1998) - et al.
Juvenile polyarteritis: results of a multicenter survey of 110 children
J Pediatr
(2004) - et al.
Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients
Semin Arthritis Rheum
(2009) - et al.
Advances in the treatment of small vessel vasculitis
Rheum Dis Clin North Am
(2006) - et al.
EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides
Ann Rheum Dis
(2006)
The childhood arthritis & rheumatology research alliance network registry: demographics and characteristics of the initial 6-month cohort
Demography of a regional pediatric rheumatology patient population. Affiliated Children’s Arthritis Centers of New England
J Rheumatol
The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry
J Rheumatol
Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. Pediatric Rheumatology Database Research Group
J Rheumatol
EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria
Ann Rheum Dis
Increased frequency of C4B*Q0 alleles in patients with Henoch-Schönlein purpura
Scand J Immunol
Temporal association of Streptococcus, Staphylococcus, and parainfluenza pediatric hospitalizations and hospitalized cases of Henoch-Schönlein purpura
J Rheumatol
Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature
Medicine (Baltimore)
Henoch-Schönlein purpura
Curr Opin Rheumatol
Risk of long term renal impairment and duration of follow up recommended for Henoch-Schönlein purpura with normal or minimal urinary findings: a systematic review
Arch Dis Child
Long-term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population
Eur J Pediatr
Epidemiology of Henoch-Schönlein purpura
Cleve Clin J Med
Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schönlein purpura
Pediatrics
Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review
Pediatrics
Renal manifestations of Henoch-Schönlein purpura in a 6-month prospective study of 223 children
Arch Dis Child
Hospitalizations for Kawasaki syndrome among children in the United States, 1997–2007
Pediatr Infect Dis J
Results of the nationwide epidemiologic survey of Kawasaki disease in 1995 and 1996 in Japan
Pediatrics
Epidemiologic features of Kawasaki disease in Taiwan, 2003–2006
Pediatrics
The potential role of cytokine-mediated vascular endothelial activation in the pathogenesis of Kawasaki disease
Acta Paediatr Jpn
Cited by (98)
Kawasaki Disease in the Australian Population: An Australian Tertiary Hospital Experience
2021, Heart Lung and CirculationCitation Excerpt :Kawasaki disease (KD) is the second commonest childhood vasculitis [1] and the leading cause of acquired heart disease among children in developed countries [2].
Systematic review of childhood-onset polyarteritis nodosa and DADA2
2021, Seminars in Arthritis and RheumatismPulmonary Manifestations of Systemic Vasculitis in Children
2021, Pediatric Clinics of North AmericaTales from the Night:: Emergency MR Imaging in Pediatric Patients after Hours
2019, Magnetic Resonance Imaging Clinics of North AmericaCitation Excerpt :In the nonfebrile, subacute phase of the illness, children are more likely to develop aneurysms, and are at the highest risk for sudden death.82,83 The convalescent phase of KD may last months to years.84 The presentation of TA depends on the arterial distribution.
Kidney Manifestations of Rheumatological Diseases in Children
2023, Current Treatment Options in PediatricsDemographic and clinical features of pediatric vasculitis: a single-center study
2023, ARP Rheumatology
Disclosures: None.