Abstract
Systemic vasculitis is a group of disorders with multiorgan involvement. These disorders have diverse clinical manifestations associated with significant morbidity and mortality. The most common vasculitides in children—Henoch-Schönlein purpura and Kawasaki disease—are self-limiting conditions. The lifelong and chronic vasculitides (eg, giant cell arteritis, Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, and Takayasu arteritis) are rarely seen in children. Therefore, the outcome in general is more favorable in children. This article offers an overview of the epidemiologic, etiologic, pathophysiologic, and clinical features of vasculitis in children, with emphasis on common conditions.
Similar content being viewed by others
References and Recommended Reading
Dillon MJ, Ozen S: A new international classification of childhood vasculitis. Pediatr Nephrol 2006, 21:1219–1222.
Ozen S, Ruperto N, Dillon MJ, et al.: EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006, 65:936–941.
Gedalia A: Henoch-Schönlein purpura. Curr Rheumatol Rep 2004, 6:195–202.
Tizard EJ, Hamilton-Ayres MJ: Henoch-Schönlein purpura. Arch Dis Child Ed Pract 2008, 93:1–8.
Mills JA, Michel BA, Bloch DA, et al.: The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 1990, 33:1114–1121.
Shetty AK, Dessele BC, Ey JL, et al.: Infantile Henoch-Schönlein purpura. Arch Fam Med 2000, 9:553–556.
Lau KK, Wayett RJ, Moldoveanu Z, et al.: Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schönlein purpura. Pediatr Nephrol 2007, 22:2067–2072.
Fujieda M, Oishi N, Naruse K, et al.: Soluble thrombomodulin and antibodies to bovine glomerular endothelial cells in patients with Henoch-Schönlein purpura. Arch Dis Child 1998, 78:240–244.
Fessatou S, Nicolaidou P, Gourgiotis D, et al.: Endothelin 1 levels in relation to clinical presentation and outcome of Henoch-Schönlein purpura. BMC Pediatr 2008, 8:33.
Brogan PA: What’s new in the aetiopathogenesis of vasculitis? Pediatr Nephrol 2007, 22:1083–1094.
Karreman M, Jordan AJ, Bell N et al.: Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature. Clin Pediatr 2009, 48:323–326.
Huber Am, King J, McLaine P, et al.: A randomized, placebo-controlled trial of prednisone in early Henoch-Schönlein purpura (ISRCTN85109383). BMC Med 2004, 2:7.
Ronkainen J, Koskimeies O, la-Houhala M, et al.: Early prednisone therapy in Henoch-Schönlein purpura: a randomized double-blind, placebo-controlled trial. J Pediatr 2006, 149:241–247.
Singh S, Devidayal, Kumar L, et al.: Severe Henoch-Schönlein nephritis: resolution with azathioprine and steroids. Rheumatol Int 2002, 22:133–137.
Shin JI, Park JM, Lee JS, et al.: Successful use of cyclosporine A in severe Schönlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine. Clin Rheumatol 2006, 25:759–760.
Weiss PF, Feinstein JA, Luan X, et al.: Effects of corticosteroid on Henoch-Schönlein purpura nephritis: a systematic review. Pediatr 2007, 120:1079–1087.
Kawasaki Y, Suzuki J, Nozawa R, et al.: Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch-Schönlein nephritis. Pediatrics 2003, 785–789.
Chartapisak W, Opastirakul S, Willis N, et al.: Prevention and treatment of renal disease in Henoch-Schonlein purpura: a systematic review. Arch Dis Child 2009, 94:132–137.
Lamireau T, Rebouissoux L, Hehunstre JP: Intravenous immunoglobulin therapy for severe digestive manifestations of Henoch-Schönlein purpura. Acta Pediatr 2001, 90:1081–1082.
Shenoy M, Ognjanovic MV, Coulthard MG: Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol 2007, 22:1167–1171.
Kawasaki Y, Suzuki J, Sakai N, et al.: Clinical and pathological features of children with Henoch-Schönlein nephritis: risk factors associated with poor prognosis. Clin Nephrol 2003, 60:153–160.
Narchi H: 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 2005, 90:916–920.
Newberger JW, Takahashi M, Gerber MA, et al.: Endorsed clinical report, AAP, AHA: guidance for the clinicians in rendering pediatric care. Pediatrics 2004, 114:1708–1733.
Freeman AF, Shulman ST: Kawasaki disease: summary of the American Heart Association Guidelines. Am Fam Physicians 2006, 74:1141–1148.
Yanagawa H, Nakamura Y, Yashiro M, et al.: Incidence of Kawasaki disease in Japan: the nationwide survey of 1999–2002. Pediatr Int 2006, 48:356–361.
Suenaga T, Suzuki H, Shibuta S, et al.: Detection of multiple superantigen genes in stool of patients with Kawasaki syndrome. Pediatr 2009, 155:266–270.
Rowley AH, Shulman ST: New developments in the search for etiologic agent of Kawasaki disease. Curr Opin Pediatr 2007, 19:71–74.
Takahashi K, Oharaseki T, Naoe S, et al.: Neutrophilic involvement in the damage to coronary arteries in acute stage of Kawasaki disease. Pediatr Int 2005, 47:305–310.
Senzaki H: The pathophysiology of coronary artery aneurysms in Kawasaki disease: role of matrix metalloproteinases. Arch Dis Child 2006, 91:847–851.
Abuhammour WM, Hasan RA, Eljamal A, Asmar B: Kawasaki disease hospitalizations in a predominantly African-American population. Clin Pediatr 2005, 44:721–725.
Porcalla AR, Sable CA, Patel KM, et al.: The epidemiology of Kawasaki disease in an urban hospital: does African American race protect against coronary artery aneurysms? Pediatr Cardiol 2005, 26:775–781.
Marquez J, Gedalia O, Candia L, et al.: Kawasaki disease: clinical spectrum of 88 patients in a high prevalence African American population. J Natl Med Assoc 2007, 100:28–32.
Kavey RE, Allada V, Daniels SR, et al.: Cardiovascular risk reduction in high-risk pediatric patients: a scientific statement from the AHA expert panel on population and prevention science. Circulation 2006, 114:2710–2738.
Hata A, Onouchi Y: Susceptibility genes for Kawasaki disease: toward implementation of personalized medicine. J Hum Genet 2009, 54:67–73.
Dahdah N, Siles A, Fournier A, et al.: Natriuretic peptide as an adjunctive diagnostic test in the acute phase of Kawasaki Disease. Pediatr Cardiol 2009, 30:810–817.
Inoue Y, Okada Y, Shinohara M, et al.: Multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr 2006, 149:336–341.
Newburger JW, Sleeper LA, McCrindle BW, et al.: Randomized trial of pulsed corticosteroids therapy for primary for treatment of Kawasaki disease. N Engl J Med 2007, 356:663–675.
Burns JC, Best BM, Mejias A, et al.: Infliximab treatment for intravenous immunoglobulin-resistant Kawasaki disease. J Pediatr 2008, 153:833–838.
Hirono K, Kemmotsu Y, Wittkowski H, et al.: Infliximab reduces the cytokine-mediated inflammation but does not suppress cellular infiltration of the vessel wall in with refractory Kawasaki Disease. Pediatr Res 2009, 65:696–701.
Brogan RJ, Eleftheriou D, Gnanapragasam J, et al.: Infliximab for the treatment of intravenous immunoglobulin resistant Kawasaki disease complicated by coronary artery aneurysms: a case report. BMC Pediatr Rheumatol 2009, 7:3.
Yalçinkaya F, Ozçakar ZB, Kasapçopur O, et al.: Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa. J Pediatr 2007, 151:675–678.
Dedeoglu F, Sundel RP: Vasculitis in children. Rheum Dis Clin N Am 2007, 33:555–583.
de Kort SW, van Rossum MA, ten Juvy CR: Infliximab in a child with therapy-resistant systemic vasculitis. Clin Rheumatol 2006, 25:769–771.
Sonomoto K, Miyamura T, Watanabe H, et al.: A case of polyarteritis nodosa successfully treated by rituximab [in Japanese]. Nihon Rinsho Meneki Gakkai Kaishi 2008, 31:119–123.
Ozen S, Bakkaloglu A, Dusunsel R, et al.: Turkish Pediatric Vasculitis Study Group: childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 2007, 26:196–200.
Al Abrawi S, Fouillet-Desjonqueres M, David L, et al.: Takayasu arteritis in children. Pediatr Rheumatol Online 2008, 6:17.
Cakar N, Yalcinkaya F, Duzova A, et al.: Takayasu’s arteritis in children. J Rheumatol 2008, 35:913–919.
Aluquin VP, Albano SA, Chan F, et al.: Magnetic resonance imaging in the diagnosis and follow up of Takayasu arteritis in children. Ann Rheum Dis 2002, 61:526–529.
Shetty AK, Stopa AR, Gedalia A: Low dose methotrexate as a steroid-sparing agent in a child with Takayasu arteritis. Clin Exp Rheumatol 1998, 16:335–336.
Filocamo G, Buoncompagni A, Viola S, et al.: Treatment of Takayasu’s arteritis with tumor necrosis factor antagonists. J Pediatr 2008, 153:432–434.
Elbers J, Benseler SM: Central nervous system vasculitis in children. Curr Opin Rheumatol 2008, 20:47–54.
Cantez S, Benseler S: Childhood CNS vasculitis: a treatable cause of neurological deficit in children. Nat Clin Pract Rheumatol 2008, 4:460–461.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Gedalia, A., Cuchacovich, R. Systemic vasculitis in childhood. Curr Rheumatol Rep 11, 402–409 (2009). https://doi.org/10.1007/s11926-009-0059-4
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11926-009-0059-4