Aggiornamento
Le vasculiti “maggiori” in età pediatrica
“MAJOR” VASCULITIDES IN CHILDHOOD
Matteo Pavan, Federico Marchetti, Loredana Lepore
Clinica Pediatrica, IRCCS “Burlo Garofolo”, Trieste
Aprile 2011 - pagg. 223 -228
Abstract
The most common vasculitides in children are Schönlein-Henoch purpura and Kawasaki disease,
both of which are usually easy to diagnose and treat and have a good prognosis.
Nevertheless, the typical adult vasculitides, such as Takayasu arteritis, Wegener granulomatosis,
Churg-Strauss syndrome and cutaneous polyarteritis can also occur in childhood.
Because of the very low incidence of these conditions in children, suspecting them is difficult
and diagnostic delay is frequent. This can lead to severe organ damage often associated
with a poor prognosis. Some simple suggestions applied to the daily clinical practice
can be helpful in recognizing these conditions and making an early diagnosis in order
to begin a prompt therapy. Despite this, the treatment of these vasculitides is difficult
and complete remission is not always achieved although it usually improves the long term
prognosis.
Parole chiave
Classificazione MeSH
Bibliografia
1. Ozen S, Fuhlbrigge RC. Update in Pediatric
Vasculitis. Best Pract Res Clin Rheumatol
2009;23:679-88.
2. Lane SE, Watts RA, Bentham G, et al. Are
Environmental Factors Important in Primary
Systemic Vasculitis? Arthritis Rheum 2003;3:
814-23.
3. Sneller MC, Langford CA, Fauci AS. The Vasculitis
Syndrome. Harrison’s Principles of Internal
Medicine, 16th ed, pp 2002-14. Mc-
Graw-Hill Professional, 2004.
4. Kallenberg CG. Pathophysiology of ANCAassociated
small vessel vasculitis. Curr Rheumatol
Rep 2010;12:399-405.
5. 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.
6. Ruperto N, Ozen S, Pistorio A, et al. EULAR/
PRINTO/PRES criteria for Henoch-
Schönlein purpura, childhood polyarteritis nodosa,
childhood Wegener granulomatosis and
childhood Takayasu arteritis: Ankara 2008.
Part I: Overall methodology and clinical characterization.
Ann Rheum Dis 2010;69:790-7.
7. Ozen S, Pistorio A, Iusan SM, et al. EULAR/
PRINTO/PRES criteria for Henoch-
Schonlein purpura, childhood polyarteritis nodosa,
childhood Wegener granulomatosis and
childhood Takayasu arteritis: Ankara 2008.
Part II: Final classification criteria. Ann
Rheum Dis 2010;69:798-806.
8. Lindsley CB, Laxer RM. Granulomatous vasculitis,
giant cell arteritis, and sarcoidosis. In:
Cassidy JT & Petty RE (Eds). Textbook of Pediatric
Rheumatology, 5th ed., pp 539-58. Philadelphia:
WB Sonders Co, 2005.
9. Vanoli M, Daina E, Salvarani C, et al.
Takayasu arteritis: a study of 104 Italian patients.
Arthritis Rheum 2005;53:100-7.
10. Cakar N, Yalcinkaya F, Duzova A, et al.
Takayasu arteritis in children. J Rheumatol
2008;35:913-9.
11. Hoffman GS, Merkel PA, Brasington RD,
et al. Anti-tumor necrosis factor therapy in patients
with difficult to treat Takayasu arteritis.
Arthritis Rheum 2004;50:2296-304.
12. Reddy E, Robbs JV. Surgical management
of Takayasu’s arteritis in children and adolescent.
Cardiovasc J Afr 2007;18:393-6.
13. Klemmer PJ, Chalermskulrat W, Reif MS,
Hogan SL, Henke DC, Falk RJ. Plasmapheresis
therapy for diffuse alveolar hemorrhage in
patients with small-vessel vasculitis. Am J Kidney
Dis 2003;42:1149-53.
14. Jobe AH. Pulmonary surfactant therapy. N
Eng J Med 1993;328:861-8.
15. Willson DF, Thomas NJ, Markovitz BP, et
al. Effect of exogenous surfactant (calfactant)
in pediatric acute lung injury: a randomized
controlled trial. JAMA 2005;293:470-6.
16. Stone JH, Merkel PA, Spiera R, et al. Rituximab
versus cyclophosphamide for ANCAassociated
vasculitis. N Eng J Med 2010;363:
221-32.
17. Gardner-Medwin JM, Dolezalova P, Cummins
C, Southwood TR. Incidence of Henoch-
Schönlein purpura, Kawasaki disease, and
other vasculitides in children of different ethnic
origin. Lancet 2002;360:1197-202.
18. Rottem M, Fauci AS, Hallahan CW, et al.
Wegener’ granulomatosis in children and adolescent:
clinical presentation and outcome. J
Pediatr 1993;122:26-31.
19. Ozaltin F, Bakkaloglu A, Ozen S, et al. The
significance of IgA class of antineutrophil citoplasmyc
antibodies (ANCA) in childhood
Henoch-Schönlein purpura. Clin Rheumatol
2004;23:426-9.
20. Boyer D, Vargas SO, Slattery D, Rivera-
Sanchez YM, Colin AA. Churg-Strauss syndrome
in children: a clinical and pathology review.
Pediatrics 2006;118:914-20.
21. Hellmich B, Gross W. Recent progress in
the pharmacotherapy of Churg-Strauss syndrome.
Expert Opin Pharmacother 2004;5:25-
35.
22. Zwerina J, Eger g, Englbrecht M, Manger
B, Schett G. Churg-Strauss syndrome in
childhood: a systematic literature review and
clinical comparison with adult patients. Semin
Arthritis Rheum 2009;39:108-15.
23. Fathalla BM, Miller L, Brady S, Schaller
JG. Cutaneous polyarteritis in children. J Am
Acad Dermatol 2005;53:724-8.
24. Morgan AJ, Schwartz RA. Cutaneous
polyarteritis nodosa: a comprehensive review.
Int J Dermatol 2010;49:750-6.
25. Mazokopakis EE, Milkas AN, Tsrtsalis
AN, Karefilakis CM, Besmertis LP. Improvement
of cutaneous polyarteritis nodosa lesions
with hyperbaric oxygen. Int J Dermatol
2009;48:1023-5.
26. Booth AD, Jayne DR, Kharbanda RK, et al.
Infliximab improves endothelial dysfunction
in systemic vasculitis. Circulation 2004;109:
1718-23.
Corrispondenza: leporel@burlo.trieste.it