Abstract
Based on surverys of providers, vasculitis in children appears to have an incidence of about 50 cases per 100,000 children per year. Only HSP and KD are more common in children than in adults, with each one affecting at least 10–20/100,000 pediatric patients per year. Takayasu arteritis and ANCA-positive vasculiti-des, on the other hand, strike approximately one child in a million each year. Differences between adult and pediatric vasculitides are generally subtle, though they may have important implications. Since these conditions are so rare in the pediatric age group, diagnosis may be significantly delayed, leading to more disease-related damage at the time therapy is initiated. In addition, unlike adults, children may suffer irreversible effects on their physical and mental development both from the disease and from the medications used to treat it. On the other hand, children tend to have fewer comor-bidities and they are therefore more tolerant of potentially toxic therapies. This often results in better outcomes for children than adults, especially in conditions like Takayasu arteritis and PAN. Kawasaki disease (KD) has replaced acute rheumatic fever as the leading cause of acquired heart disease among children in developed nations. Since IVIG became the standard treatment for KD in 1986, the mortality rate of the disease has decreased from 1–2% to <0.1%, and the incidence of coronary artery aneu-rysms has decreased by 75%. Henoch-Schonlein purpura, also known as anaphylac-tic purpura, is a benign condition in the vast majority of affected children. Nonetheless, in rare cases, children affected by HSP may develop hypertension (especially during pregnancy) or renal failure long after the acute syndrome has resolved. One-third or more of children with PAN have the cutaneous form, largely limited to skin and joint involvement. While mortality is rare, aggressive control of the systemic inflammation and end-organ involvement is nonetheless needed if significant long-term morbidity is to be avoided.
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References
Allen AC, Willis FR, Beattie TJ, et al. Abnormal IgA glycosylation in Henoch–Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant. 1998;13:930–4
Amano S, Hazama F, Kubagawa H, et al. General pathology of Kawasaki disease. Acta Pathol Jpn. 1980;30(5):681–94
Anderson MS, Burns J, Treadwell TA, et al. Erythrocyte sedimentation rate and C-reactive protein discrepancy and high prevalence of coronary artery abnormalities in Kawasaki disease. Pediatr Infect Dis J. 2001;20(7):698–702
Bártfai Z, Gaede KI, Russell KA, et al. Different gender-associated genotype risks of Wegener's granulomatosis and microscopic poly-angiitis. Clin Immunol. 2003;109(3):330–7
Binstadt BA, Levine JC, Nigrovic PA, et al. Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis. Pediatrics 2005;116:e89–93
Birchmore D, Sweeney C, Choudhury D, et al. IgA multiple myeloma presenting as HSP/Polyarteritis nodosa overlap. Arthritis Rheum. 1996;39:698–703
Burns JC, Wiggins JW, Toews WH, et al. Clinical spectrum of Kawasaki disease in infants younger than 6 months of age. J Pediatr. 1986;109:759–63
Burns JC, Mason WH, Glode MP, et al. Clinical and epidemiologic characteristics of patients referred for evaluation of possible Kawasaki disease. United States Multicenter Kawasaki Disease Study Group. J Pediatr. 1991;118:680–6
Burns JC, Shimizu C, Shike H, et al. Family-based association analysis implicates IL-4 in susceptibility to Kawasaki disease. Genes Immun. 2005;6(5):438–44
Cakar N, Yalcinkaya F, Duzova A, et al. Takayasu arteritis in children. J Rheumatol. 2008;35(5):913–19
Centers for Disease Control. Haemophilus influenzae invasive disease among children age <5 years–California, 1990–1996. MMWR. 1998;47:737–40
Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health. 1998;34:405–9
Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clini-copathological study of 79 cases. Br J Dermatol. 1997;136:706–13
Dengler LD, Capparelli E V, Bastian JF, et al. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17:478–81
Frosch M, Foell D. Wegener granulomatosis in childhood and adolescence. Eur J Pediatr. 2004;163:425–34
Golding RL, Perri G, Cremin BJ. The arteriographic manifestations of Takayasu's arteritis in children. Pediatr Radiol. 1977;5:224–30
Goldstein AR, White RHR, Akuse R, et al Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 1992;339:280–2
Holman RC, Belay ED, Curns AT, et al. Kawasaki syndrome hospital-izations among children in the United States, 1988–1997. Pediatrics 2003;111:e448
Jurado RL. Why shouldn't we determine the erythrocyte sedimentation rate? Clin Infect Dis. 2001;33:548–9
Kumar L, Thapa BR, Sarkar B, Walia BN. Benign cutaneous PAN in children below 10 years of age: a clinical experience. Ann Rheum Dis. 1995;54:134–6
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;59:651–62
Momenah T, Sanatani S, Potts J, et al. Kawasaki disease in the older child. Pediatrics 1998;e102(1):e7
Muta H, Ishii M, Sakaue T, et al Older age is a risk factor for the development of cardiovascular sequelae in Kawasaki disease. Pediatrics 2004;114:751–4
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;90:916–20
Newburger J, et al Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, Am Heart Assoc Pediatr. 2004a;114:1708–33
Newburger JW, Sanders SP, Burns JC, et al Left ventricular contractility and function in Kawasaki syndrome. Circulation 1989;79:1237–46
Newburger JW, Takahashi M, Gerber MA, et al Diagnosis, treatment, and long-term management of Kawasaki disease. Circulation 2004a;110:2747–71
Niwa K, Aotsuka H, Hamada H, et al. Thrombocytopenia: a risk factor for acute myocardial infarction during the acute phase of Kawasaki Disease. Coron Artery Dis. 1995;6(11):857–64
Ozdogan H, Arisoy N, Kasapcapur O, et al. Vasculitis in familial Mediterranean fever. J Rheumatol. 1997;24:323–7
Ozen S, Bakkaloglu A, Yilmaz E, Duzova A, Balci B, Topaloglu R, Besbas N. Mutations in the gene for familial Mediterranean fever: do they predispose to inflammation? J Rheumatol. 2003;30(9):2014–8
Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145:517–22
Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–41
Ozen S, Duzova A, Bakkaloglu A, et al. Takayasu arteritis in children: preliminary experience with cyclophosphamide induction and corti-costeroids followed by methotrexate. J Pediatr. 2007;150:72–6
Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch–Schönlein purpura: a retrospective cohort study. Lancet 2002;360:666–70
Ronkainen J, Ala-Houhala M, Huttunen NP, et al. Outcome of Henoch-Schoenlein nephritis with nephrotic-range proteinuria. Clin Nephrol. 2003;60:80–4
Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149:241–7
Rosenfeld EA, Corydon KE, Shulman ST. Kawasaki disease in infants less than one year of age. J Pediatr. 1995;126:524–49
Rottem M, Fauci AS, Hallahan CW, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. 1993;122(1):26–31
Saulsbury FT. Corticosteroid therapy does not prevent nephritis in Henoch–Schönlein purpura. Pediatr Nephrol. 1993;7:69–71
Saulsbury FT. Alterations in the O-linked glycosylation of IgA1 in children with Henoch-Schönlein purpura. J Rheumatol. 1997;24:2246–9
Saulsbury FT. Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine 1999;78:395–409
Saulsbury FT. Henoch-Schönlein purpura. Curr Opin Rheumatol. 2001;13:35–40
Seo P, Luqmani R, Flossmann O, et al. The future damage assessment in vasculitis. J Rheumatol. 2007;34:1357–71
Spriewald BM, Witzke O, Wassmuth R, et al. Distinct tumour necrosis factor alpha, interferon gamma, interleukin 10, and cytotoxic T cell antigen 4 gene polymorphisms in disease occurrence and end stage renal disease in Wegener's granulomatosis. Ann Rheum Dis. 2005;64:457–61
Stamos JK, Corydon K, Donaldson J, Shulman ST. Lymphadenitis as the dominant manifestation of Kawasaki disease. Pediatrics 1994;93(3):525–8
Stockheim JA, Innocentini N, Shulman ST. Kawasaki disease in older children and adolescents. J Pediatr. 2000;137:250–2
Stone JH, Nousari HC. Essential cutaneous vasculitis: everything a rheumatologist needs to know about vasculitis of the skin. Curr Opin Rheumatol. 2001;13:23–34
Tanaka N, Sekimoto K, Naoe S. Kawasaki disease. Relationship with infantile periarteritis nodosa. Arch Pathol Lab Med. 1976;100:81–6
Trapani S, Micheli A, Grisolia F, et al. Henoch-Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of the literature. Semin Arthritis Rheum. 2005;35:143–53
Tse W Y, Cockwell P, Savage CO. Assessment of disease activity in systemic vasculitis. Postgrad Med J. 1998;74:1–6
Umezawa T, Saji T, Matsuo N, Odagiri K. Chest X-ray findings in the acute phase of Kawasaki disease. Pediatr Radiol. 1989;20(1–2):48–51
van Leeuwen MA, van Rijswijk MH. Acute phase proteins in the monitoring of inflammatory disorders. Baillieres Clin Rheumatol. 1994;8(3):531–52
Wickiser JE, Saulsbury FT. Henoch-Schönlein purpura in a child with hyperimmunoglobulinemia D and periodic fever syndrome. Pediatr Dermatol. 2005;22:138–41
Yalçinkaya F, Ozçakar ZB, Kasapçopur O, et al. Prevalence of the MEFV gene mutations in childhood PAN. J Pediatr. 2007;151:675–8
Yanagawa H, Nakamura Y, Yashiro M, et al. Incidence survey of Kawasaki disease in 1997 and 1998 in Japan. Pediatrics 2001; 107(3):e33
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Rowley, A.H., Ozen, S., Sundel, R.P., Saulsbury, F.T. (2009). Pediatric Vasculitis. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_21
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