Vasculitis in children: Classification and incidence
- David Cabral, MBBS, FRCPC
David Cabral, MBBS, FRCPC
- Clinical Professor of Pediatrics, Head, Division of Rheumatology, Department of Pediatrics
- University of British Columbia
- Kimberly Morishita, MD, MHSc, FRCPC
Kimberly Morishita, MD, MHSc, FRCPC
- Clinical Assistant Professor of Pediatrics
- Division of Rheumatology, Department of Pediatrics
- University of British Columbia
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 .
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 . The two most common vasculitides were Henoch-Schönlein purpura (immunoglobulin A [IgA] vasculitis) 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.
- Dedeoglu F, Sundel RP. Vasculitis in children. Rheum Dis Clin North Am 2007; 33:555.
- Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002; 360:1197.
- Dolezalová P, Telekesová P, Nemcová D, Hoza J. Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. J Rheumatol 2004; 31:2295.
- Brogan PA, Dillon MJ. Vasculitis from the pediatric perspective. Curr Rheumatol Rep 2000; 2:411.
- Wilkinson NM, Page J, Uribe AG, et al. Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey. J Rheumatol 2007; 34:224.
- Hunder GG, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum 1990; 33:1065.
- Rao JK, Allen NB, Pincus T. Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med 1998; 129:345.
- Watts RA, Suppiah R, Merkel PA, Luqmani R. Systemic vasculitis--is it time to reclassify? Rheumatology (Oxford) 2011; 50:643.
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
- Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
- Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337:1512.
- Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry. J Rheumatol 1996; 23:1981.
- Bowyer S, Roettcher P. Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. Pediatric Rheumatology Database Research Group. J Rheumatol 1996; 23:1968.
- Basu N, Watts R, Bajema I, et al. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann Rheum Dis 2010; 69:1744.
- Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006; 65:936.
- Ozen S, Pistorio A, Iusan SM, et al. 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 2010; 69:798.
- Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi 1967; 16:178.
- Navon Elkan P, Pierce SB, Segel R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med 2014; 370:921.
- ROBERTS FB, FETTERMAN GH. POLYARTERITIS NODOSA IN INFANCY. J Pediatr 1963; 63:519.
- Ettlinger RE, Nelson AM, Burke EC, Lie JT. Polyarteritis nodosa in childhood a clinical pathologic study. Arthritis Rheum 1979; 22:820.
- Engel DG, Gospe SM Jr, Tracy KA, et al. Fatal infantile polyarteritis nodosa with predominant central nervous system involvement. Stroke 1995; 26:699.
- Landing BH, Larson EJ. Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same? Comparison of 20 patients from North America with patients from Hawaii and Japan. Pediatrics 1977; 59:651.
- Bowyer S, Mason WH, McCurdy DK, Takahashi M. Polyarteritis nodosa (PAN) with coronary aneurysms: the kawasaki-PAN controversy revisited. J Rheumatol 1994; 21:1585.
- David J, Ansell BM, Woo P. Polyarteritis nodosa associated with streptococcus. Arch Dis Child 1993; 69:685.
- Fink CW. The role of the streptococcus in poststreptococcal reactive arthritis and childhood polyarteritis nodosa. J Rheumatol Suppl 1991; 29:14.
- Till SH, Amos RS. Long-term follow-up of juvenile-onset cutaneous polyarteritis nodosa associated with streptococcal infection. Br J Rheumatol 1997; 36:909.
- Sheth AP, Olson JC, Esterly NB. Cutaneous polyarteritis nodosa of childhood. J Am Acad Dermatol 1994; 31:561.
- Brunner J, Feldman BM, Tyrrell PN, et al. Takayasu arteritis in children and adolescents. Rheumatology (Oxford) 2010; 49:1806.
- Cabral DA, Uribe AG, Benseler S, et al. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis Rheum 2009; 60:3413.
- Cotch MF, Hoffman GS, Yerg DE, et al. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 1996; 39:87.
- Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis Rheum 2000; 43:2481.
- Stegmayr BG, Gothefors L, Malmer B, et al. Wegener granulomatosis in children and young adults. A case study of ten patients. Pediatr Nephrol 2000; 14:208.
- Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66:222.
- Sablé-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143:632.
- Gendelman S, Zeft A, Spalding SJ. Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort. J Rheumatol 2013; 40:929.
- Benseler SM, Silverman E, Aviv RI, et al. Primary central nervous system vasculitis in children. Arthritis Rheum 2006; 54:1291.
- Cellucci T, Benseler SM. Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr 2010; 22:731.
- Benseler S, Schneider R. Central nervous system vasculitis in children. Curr Opin Rheumatol 2004; 16:43.
- Eleftheriou D, Cox T, Saunders D, et al. Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography. Dev Med Child Neurol 2010; 52:863.
- Benseler SM, deVeber G, Hawkins C, et al. Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum 2005; 52:2159.
- Zhou Q, Yang D, Ombrello AK, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med 2014; 370:911.
- Liu Y, Jesus AA, Marrero B, et al. Activated STING in a vascular and pulmonary syndrome. N Engl J Med 2014; 371:507.
- Torrelo A, Patel S, Colmenero I, et al. Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome. J Am Acad Dermatol 2010; 62:489.
- Liu Y, Ramot Y, Torrelo A, et al. Mutations in proteasome subunit β type 8 cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature with evidence of genetic and phenotypic heterogeneity. Arthritis Rheum 2012; 64:895.
- Brehm A, Liu Y, Sheikh A, et al. Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production. J Clin Invest 2015; 125:4196.
- CLASSIFICATION VERSUS DIAGNOSIS
- CLASSIFICATION SYSTEMS
- CLASSIFICATION CRITERIA: MAJOR PRIMARY VASCULITIDES
- Henoch-Schönlein purpura (IgA vasculitis)
- Kawasaki disease
- Polyarteritis nodosa
- Infantile polyarteritis nodosa
- Cutaneous polyarteritis nodosa
- Takayasu arteritis
- Granulomatosis with polyangiitis (Wegener's)
- OTHER CHILDHOOD PRIMARY VASCULITIDES
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
- Hypersensitivity vasculitis
- Behçet's syndrome
- Primary angiitis of the central nervous system
- SECONDARY VASCULITIS
- MONOGENIC VASCULITIS
- SOCIETY GUIDELINE LINKS