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Medium-size-vessel vasculitis

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Abstract

Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.

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References

  1. Tervaert JW, Kallenberg C (1993) Neurologic manifestations of systemic vasculitides. Rheum Dis Clin North Am 19:913–940

    CAS  PubMed  Google Scholar 

  2. Jennette JC, Falk RJ (1997) Small-vessel vasculitis. N Engl J Med 337:1512–1523

    Article  CAS  PubMed  Google Scholar 

  3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, McClusky RT, Sinico A, Rees AJ, vanEs LA, Waldherr R, Wiik A (1994) Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 37:187–192

    Article  CAS  PubMed  Google Scholar 

  4. Savage CO, Harper L, Adu D (1997) Primary systemic vasculitis. Lancet 349:553–558

    Article  CAS  PubMed  Google Scholar 

  5. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, Kawasaki T, Lindsley C, Petty RE, Prieur AM, Ravelli A, Woo P (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 65:936–941

    Article  CAS  PubMed  Google Scholar 

  6. Fink CW (1986) Vasculitis. Pediatr Clin North Am 33:1203–1219

    CAS  PubMed  Google Scholar 

  7. Dillon MJ (1998) Childhood vasculitis. Lupus 7:259–265

    Article  CAS  PubMed  Google Scholar 

  8. Lie JT (1994) Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose. Arthritis Rheum 37:181–186

    Article  CAS  PubMed  Google Scholar 

  9. Cassidy JT, Petty RE (2005) Polyarteritis nodosa and related vasculitides. In: Cassidy JT, Petty RE (eds) Textbook of pediatric rheumatology, 5th edn. Elsevier Saunders, Philadelphia, pp 512–552

    Google Scholar 

  10. Kussmaul A, Maier R (1866) Über eine bisher nicht beschriebene eigenthümliche Arterienerkrankung (Periarteriitis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1:484–518

    Google Scholar 

  11. Watts RA, Scott DGI (2008) Epidemiology of vasculitis. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 7–21

    Google Scholar 

  12. Dillon MJ (1989) Classification and pathogenesis of arteritis in children. Toxicol Pathol 17:214–218

    CAS  PubMed  Google Scholar 

  13. Ozen S, Bakkaloglu A, Dusunsel R, Soylemezoglu O, Ozaltin F, Poyrazoglu H, Kasapcopur O, Ozkaya O, Yalcinkaya F, Balat A, Kural N, Donmez O, Alpay H, Anarat A, Mir S, Gur-Guven A, Sonmez F, Gok F (2007) Childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 26:196–200

    PubMed  Google Scholar 

  14. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, Arend WP, Calabrese LH, Leavitt RY, Lie JT, Masi AT, Mitts JH, Stevens MB, Wallace SL (1990) The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 33:1088–1093

    PubMed  Google Scholar 

  15. Dillon MJ, Ozen S (2006) A new international classification of childhood vasculitis. Pediatr Nephrol 21:1219–1222

    Article  PubMed  Google Scholar 

  16. Ozen S, Pistorio A, Iusan MS, Ravelli A, Cuttica R, Lovell DJ, Anton J, Bilge I, Garay SM, Saad Magalhaes C, Kondi A, Bircan Z, Breda L, Cimaz R, Kanakoudi Tsakalidou F, Magni Manzoni S, Maldonado MR, Stanevicha V, Tambic-Bukovac L, Unsal E, Martini A, Ruperto N, for PRINTO (2009) The EULAR/PRINTO/PRES criteria for childhood polyarteritis nodosa. Ankara 2008. Ann Rheum Dis 68(Suppl 3):713

    Google Scholar 

  17. Hunder GG (1998) The use and misuse of classification and diagnostic criteria for complex diseases. Ann Intern Med 129:417–418

    CAS  PubMed  Google Scholar 

  18. Ozen S, Besbas N, Saatci U, Bakkaloglu A (1992) Diagnostic criteria for polyarteritis nodosa in childhood. J Pediatr 120:206–209

    Article  CAS  PubMed  Google Scholar 

  19. Fink CW (1977) Polyarteritis and other diseases with necrotizing vasculitis in childhood. Arthritis Rheum 20:378–384

    CAS  PubMed  Google Scholar 

  20. Reimold EW, Weinberg AG, Fink CW, Battles ND (1976) Polyarteritis in children. Am J Dis Child 130:534–541

    CAS  PubMed  Google Scholar 

  21. Magilavy DB, Petty RE, Cassidy JT, Sullivan DB (1977) A syndrome of childhood polyarteritis. J Pediatr 91:25–30

    Article  CAS  PubMed  Google Scholar 

  22. Ettlinger RE, Nelson AM, Burke EC, Lie JT (1979) Polyarteritis nodosa in childhood a clinical pathologic study. Arthritis Rheum 22:820–825

    Article  CAS  PubMed  Google Scholar 

  23. Maeda M, Kobayashi M, Okamoto S, Fuse T, Matsuyama T, Watanabe N, Fujikawa S (1997) Clinical observation of 14 cases of childhood polyarteritis nodosa in Japan. Acta Paediatr Jpn 39:277–279

    CAS  PubMed  Google Scholar 

  24. Eleftheriou D, Dillon MJ, Brogan PA (2009) Advances in childhood vasculitis. Curr Opin Rheumatol 21:411–418

    Article  PubMed  Google Scholar 

  25. Petty RE, Magilavy DB, Cassidy JT, Sullivan DB (1977) Polyarteritis in childhood. A clinical description of eight cases. Arthritis Rheum (Suppl 20):392–394

  26. Besbas N, Ozen S, Saatci U, Topaloglu R, Tinaztepe K, Bakkaloglu A (2000) Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children. Pediatr Nephrol 14:325–327

    Article  CAS  PubMed  Google Scholar 

  27. Cakar N, Ozcakar ZB, Soy D, Ucar Y, Fitoz S, Kara N, Uncu N, Atakan C, Ekim M, Yalcinkaya F (2008) Renal involvement in childhood vasculitis. Nephron Clin Pract 108:c202–c206

    Article  PubMed  Google Scholar 

  28. Topaloglu R, Besbas N, Saatci U, Bakkaloglu A, Oner A (1992) Cranial nerve involvement in childhood polyarteritis nodosa. Clin Neurol Neurosurg 94:11–13

    Article  CAS  PubMed  Google Scholar 

  29. Engel DG, Gospe SM Jr, Tracy KA, Ellis WG, Lie JT (1995) Fatal infantile polyarteritis nodosa with predominant central nervous system involvement. Stroke 26:699–701

    CAS  PubMed  Google Scholar 

  30. Ragge NK, Harris CM, Dillon MJ, Chong WK, Elston J, Taylor DS (2003) Ocular tilt reaction due to a mesencephalic lesion in juvenile polyarteritis nodosa. Am J Ophthalmol 135:249–251

    Article  PubMed  Google Scholar 

  31. Tizard EJ, Dillon MJ (2006) Wegener’s granulomatosis, polyarteritis nodosa, Behcet’s disease and relapsing polychondritis. In: Harper J, Oranje A, Prose N (eds) Textbook of pediatric dermatology, 2nd edn. Blackwell, Oxford, pp 1937–1952

    Google Scholar 

  32. Guillevin L, Pagnoux C, Teixeira L (2008) Polyarteritis nodosa. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 335–353

    Google Scholar 

  33. Brogan PA, Davies R, Gordon I, Dillon MJ (2002) Renal angiography in children with polyarteritis nodosa. Pediatr Nephrol 17:277–283

    Article  PubMed  Google Scholar 

  34. Dillon MJ (1990) Vasculitic syndromes. In: Woo P, White PH, Ansell BM (eds) Paediatric rheumatology update. Oxford University Press, Oxford, pp 227–242

    Google Scholar 

  35. Tasdemir I, Turgan C, Emri S, Yasavul U, Caglar S, Bakkaloglu M, Erkan I, Erol D (1988) Spontaneous perirenal haematoma secondary to polyarteritis nodosa. Br J Urol 62:219–222

    Article  CAS  PubMed  Google Scholar 

  36. Glikson M, Galun E, Schlesinger M, Cohen D, Haskell L, Rubinow A, Eliakim M (1989) Polyarteritis nodosa and familial Mediterranean fever: a report of 2 cases and review of the literature. J Rheumatol 16:536–539

    CAS  PubMed  Google Scholar 

  37. Ozen S, Ben-Chetrit E, Bakkaloglu A, Gur H, Tinaztepe K, Calguneri M, Turgan C, Turkmen A, Akpolat I, Danaci M, Besbas N, Akpolat T (2001) Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF? Semin Arthritis Rheum 30:281–287

    Article  CAS  PubMed  Google Scholar 

  38. Bywaters EG (1968) The Cheshire cat syndrome. Postgrad Med J 44:19–22

    Article  CAS  PubMed  Google Scholar 

  39. Levin M, Holland PC, Nokes TJ, Novelli V, Mola M, Levinsky RJ, Dillon MJ, Barratt TM, Marshall WC (1985) Platelet immune complex interaction in pathogenesis of Kawasaki disease and childhood polyarteritis. Br Med J (Clin Res Ed) 290:1456–1460

    Article  CAS  Google Scholar 

  40. Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, Garcia-Consuegra J, Dolezalova P, Dressler F, Duzova A, Ferriani VP, Hilario MO, Ibanez-Rubio M, Kasapcopur O, Kuis W, Lehman TJ, Nemcova D, Nielsen S, Oliveira SK, Schikler K, Sztajnbok F, Terreri MT, Zulian F, Woo P (2004) Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr 145:517–522

    Article  PubMed  Google Scholar 

  41. Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E, Cohen P (2005) Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 84:313–322

    Article  Google Scholar 

  42. Gaskin G, Savage CO, Ryan JJ, Jones S, Rees AJ, Lockwood CM, Pusey CD (1991) Anti-neutrophil cytoplasmic antibodies and disease activity during long-term follow-up of 70 patients with systemic vasculitis. Nephrol Dial Transplant 6:689–694

    CAS  PubMed  Google Scholar 

  43. Hauschild S, Csernok E, Schmitt WH, Gross WL (1994) Antineutrophil cytoplasmic antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome-62 patients. J Rheumatol 21:1173–1174

    CAS  PubMed  Google Scholar 

  44. Wong SN, Shah V, Dillon MJ (1998) Antineutrophil cytoplasmic antibodies in Wegener’s granulomatosis. Arch Dis Child 79:246–250

    Article  CAS  PubMed  Google Scholar 

  45. Bakkaloglu A, Ozen S, Baskin E, Besbas N, Gur-Guven A, Kasapcopur O, Tinaztepe K (2001) The significance of antineutrophil cytoplasmic antibody in microscopic polyangitis and classic polyarteritis nodosa. Arch Dis Child 85:427–430

    Article  CAS  PubMed  Google Scholar 

  46. Churg A (2008) Pathologic features of vasculitis. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 97–114

    Google Scholar 

  47. Curren RE, Steinberg I, Hagstrom WC (1967) Arteriovenous fistula complicating percutaneous renal biopsy in polyarteritis nodosa. Am J Med 43:465–470

    Article  Google Scholar 

  48. Basoglu T, Akpolat T, Canbaz F, Bernay I, Albayrak S, Kilic M, Danaci M (1999) Tc-99m DMSA renal scan in polyarteritis nodosa with bilateral intraparenchymal renal artery aneurysms. Clin Nucl Med 24:201–202

    Article  CAS  PubMed  Google Scholar 

  49. Ewald EA, Griffin D, McCune WJ (1987) Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol 4:952–956

    Google Scholar 

  50. Hekali P, Kajander H, Pajari R, Stenman S, Somer T (1991) Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol 32:143–148

    Article  CAS  PubMed  Google Scholar 

  51. Saddekni S, Horesh L, Leonardo R, Kasthuri S (2008) Angiography and percutaneous interventions. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 227–245

    Google Scholar 

  52. Sabater EA, Stanson AW (2008) Cross sectional imaging in vasculitis. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 247–265

    Google Scholar 

  53. Schmidt WA (2004) Use of imaging studies in the diagnosis of vasculitis. Curr Rheumatol Rep 6:203–211

    Article  PubMed  Google Scholar 

  54. Ozaki K, Miyayama S, Ushiogi Y, Matsui O (2009) Renal involvement of polyarteritis nodosa: CT and MR findings. Abdom Imaging 34:265–270

    Article  PubMed  Google Scholar 

  55. Ozcakar ZB, Yalcinkaya F, Fitoz S, Yuksel S, Acar B, Caltik A, Ekim M (2006) Polyarteritis nodosa: successful diagnostic imaging utilizing pulsed and color Doppler ultrasonography and computed tomography angiography. Eur J Pediatr 165:120–123

    Article  PubMed  Google Scholar 

  56. Brogan PA, Dillon MJ (2004) Endothelial microparticles and the diagnosis of the vasculitides. Intern Med 43:1115–1119

    Article  PubMed  Google Scholar 

  57. Brogan PA, Shah V, Brachet C, Harnden A, Mant D, Klein N, Dillon MJ (2004) Endothelial and platelet microparticles in vasculitis of the young. Arthritis Rheum 50:927–936

    Article  CAS  PubMed  Google Scholar 

  58. Erdbruegger U, Grossheim M, Hertel B, Wyss K, Kirsch T, Woywodt A, Haller H, Haubitz M (2008) Diagnostic role of endothelial microparticles in vasculitis. Rheumatology (Oxford) 47:1820–1825

    Article  CAS  Google Scholar 

  59. Woywodt A, Goldberg C, Kirsch T, de Groot K, Erdbruegger U, Haller H, Haubitz M (2006) Circulating endothelial cells in relapse and limited granulomatous disease due to ANCA associated vasculitis. Ann Rheum Dis 65:164–168

    Article  CAS  PubMed  Google Scholar 

  60. Holmen C, Elsheikh E, Stenvinkel P, Qureshi AR, Pettersson E, Jalkanen S, Sumitran-Holgersson S (2005) Circulating inflammatory endothelial cells contribute to endothelial progenitor cell dysfunction in patients with vasculitis and kidney involvement. J Am Soc Nephrol 16:3110–3120

    Article  PubMed  Google Scholar 

  61. de Groot K, Goldberg C, Bahlmann FH, Woywodt A, Haller H, Fliser D, Haubitz M (2007) Vascular endothelial damage and repair in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 56:3847–3853

    Article  PubMed  Google Scholar 

  62. Haubitz M, Woywodt A (2004) Circulating endothelial cells and vasculitis. Intern Med 43:660–667

    Article  CAS  PubMed  Google Scholar 

  63. Kawamoto A, Asahara T (2009) Vascular regeneration therapy for intractable vasculitis. Nippon Rinsho 67:612–618

    PubMed  Google Scholar 

  64. Clark LA, Hong Y, Eleftheriou D, Klein N, Brogan PA (2008) Endothelial progenitor cells and vasculogenic responses to therapy in children with primary systemic vasculitis. Pediatr Rheumatol 6(Suppl 1):S22

    Article  Google Scholar 

  65. Eleftheriou D, Clarke LA, Hong Y, Klein N, Ganesan V, Brogan PA (2008) Circulating endothelial cells and endothelial progenitor cells in childhood primary angiitis of the central nervous system. Pediatr Rheumatol 6(Suppl 1):P276

    Google Scholar 

  66. Finkel TH, Torok TJ, Ferguson PJ, Durigon EL, Zaki SR, Leung DY, Harbeck RJ, Gelfand EW, Saulsbury FT, Hollister JR (1994) Chronic parvovirus B19 infection and systemic necrotising vasculitis: opportunistic infection or aetiological agent? Lancet 343:1255–1258

    Article  CAS  PubMed  Google Scholar 

  67. Golden MP, Hammer SM, Wanke CA, Albrecht MA (1994) Cytomegalovirus vasculitis. Case reports and review of the literature. Medicine (Baltimore) 73:246–255

    CAS  Google Scholar 

  68. Pagnoux C, Cohen P, Guillevin L (2006) Vasculitides secondary to infections. Clin Exp Rheumatol 24(Suppl 4):S71–S81

    CAS  PubMed  Google Scholar 

  69. Fain O, Hamidou M, Cacoub P, Godeau B, Wechsler B, Paries J, Stirnemann J, Morin AS, Gatfosse M, Hanslik T, Belmatoug N, Bletry O, Cevallos R, Delevaux I, Fisher E, Hayem G, Kaplan G, Le HC, Mouthon L, Larroche C, Lemaire V, Piette AM, Piette JC, Ponge T, Puechal X, Rossert J, Sarrot-Reynauld F, Sicard D, Ziza JM, Kahn MF, Guillevin L (2007) Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum 57:1473–1480

    Article  PubMed  Google Scholar 

  70. Brogan PA, Shah V, Bagga A, Klein N, Dillon MJ (2003) T cell Vbeta repertoires in childhood vasculitides. Clin Exp Immunol 131:517–527

    Article  CAS  PubMed  Google Scholar 

  71. Guillevin L, Lhote F, Amouroux J, Gherardi R, Callard P, Casassus P (1996) Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: indications for the classification of vasculitides of the polyarteritis nodosa group. Br J Rheumatol 35:958–964

    Article  CAS  PubMed  Google Scholar 

  72. Ball GV, Bridges SL (2008) Pathogenesis of vasculitis. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 67–88

    Google Scholar 

  73. Leff R, Harrer WV, Baylis JC, Jackson L, Faber K (1971) Polyarteritis nodosa in two siblings. Am J Dis Child 121:67–70

    CAS  PubMed  Google Scholar 

  74. Rottem M, Cotch MF, Fauci AS, Hoffman GS (1994) Familial vasculitis: report of 2 families. J Rheumatol 21:561–563

    CAS  PubMed  Google Scholar 

  75. Mason JC, Cowie MR, Davies KA, Schofield JB, Cambridge J, Jackson J, So A, Allard SA, Walport MJ (1994) Familial polyarteritis nodosa. Arthritis Rheum 37:1249–1253

    Article  CAS  PubMed  Google Scholar 

  76. Brogan PA, Dillon MJ (2000) Vasculitis from the pediatric perspective. Curr Rheumatol Rep 2:411–416

    Article  CAS  PubMed  Google Scholar 

  77. Brogan PA, Dillon MJ (2000) The use of immunosuppressive and cytotoxic drugs in non-malignant disease. Arch Dis Child 83:259–264

    Article  CAS  PubMed  Google Scholar 

  78. Leib ES, Restivo C, Paulus HE (1979) Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med 67:941–947

    Article  CAS  PubMed  Google Scholar 

  79. Fauci AS, Katz P, Haynes BF, Wolff SM (1979) Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 301:235–238

    Article  CAS  PubMed  Google Scholar 

  80. Dillon MJ (2006) Vasculitis treatment - new therapeutic approaches. Eur J Pediatr 165:351–357

    Article  PubMed  Google Scholar 

  81. Jayne D (2003) Current attitudes to the therapy of vasculitis. Kidney Blood Press Res 26:231–239

    Article  PubMed  Google Scholar 

  82. Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, Dolezalova P, Ozen S, Pilkington C, Woo P, Klein N, Dillon MJ, Brogan PA (2009) Biologic therapy in primary systemic vasculitis of the young. Rheumatology 48:978–986

    Article  CAS  PubMed  Google Scholar 

  83. Gianviti A, Trompeter RS, Barratt TM, Lythgoe MF, Dillon MJ (1996) Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis. Arch Dis Child 75:186–190

    Article  CAS  PubMed  Google Scholar 

  84. Wright E, Dillon MJ, Tullus K (2007) Childhood vasculitis and plasma exchange. Eur J Pediatr 166:145–151

    Article  PubMed  Google Scholar 

  85. de Kort SW, van Rossum MA, ten Cate R (2006) Infliximab in a child with therapy-resistant systemic vasculitis. Clin Rheumatol 25:769–771

    Article  PubMed  Google Scholar 

  86. Sonomoto K, Miyamura T, Watanabe H, Takahama S, Nakamura M, Ando H, Minami R, Yamamoto M, Saito T, Imayama S, Hosokawa C, Suematsu E (2008) A case of polyarteritis nodosa successfully treated by rituximab. Nihon Rinsho Meneki Gakkai Kaishi 31:119–123

    PubMed  Google Scholar 

  87. Al-Bishri J, le Riche N, Pope JE (2005) Refractory polyarteritis nodosa successfully treated with infliximab. J Rheumatol 32:1371–1373

    PubMed  Google Scholar 

  88. Wu K, Throssell D (2006) A new treatment for polyarteritis nodosa. Nephrol Dial Transplant 21:1710–1712

    Article  PubMed  Google Scholar 

  89. Beimler JH, Andrassy K (2004) Cyclophosphamide treatment in systemic necrotizing vasculitis and lupus nephritis. How long? How much? Pediatr Nephrol 19:949–955

    Article  CAS  PubMed  Google Scholar 

  90. Gayraud M, Guillevin L, Le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B (2001) Long-term follow-up of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 44:666–675

    Article  CAS  PubMed  Google Scholar 

  91. Royer P, Levy M, Gagnadoux MF (1954) Glomerular nephropathies in systemic disease. In: Royer P, Habib R, Mathieu H, Broyer M (eds) Pediatric nephrology. WB Saunders, Philadelphia, pp 302–326

    Google Scholar 

  92. Brogan PA, Shah V, Dillon MJ (2002) Polyarteritis nodosa in childhood. Abstract. 10th International Vasculitis and ANCA Workshop, Cleveland

    Google Scholar 

  93. Cheung YF, Brogan PA, Pilla CB, Dillon MJ, Redington AN (2002) Arterial distensibility in children and teenagers: normal evolution and the effect of childhood vasculitis. Arch Dis Child 87:348–351

    Article  CAS  PubMed  Google Scholar 

  94. Bastian HM (2008) Cutaneous polyarteritis nodosa. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 365–372

    Google Scholar 

  95. Daoud MS, Hutton KP, Gibson LE (1997) Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol 136:706–713

    Article  CAS  PubMed  Google Scholar 

  96. Sheth AP, Olson JC, Esterly NB (1994) Cutaneous polyarteritis nodosa of childhood. J Am Acad Dermatol 31:561–566

    Article  CAS  PubMed  Google Scholar 

  97. Fathalla BM, Miller L, Brady S, Schaller JG (2005) Cutaneous polyarteritis nodosa in children. J Am Acad Dermatol 53:724–728

    Article  PubMed  Google Scholar 

  98. Kumar L, Thapa BR, Sarkar B, Walia BN (1995) Benign cutaneous polyarteritis nodosa in children below 10 years of age-a clinical experience. Ann Rheum Dis 54:134–136

    Article  CAS  PubMed  Google Scholar 

  99. Minkowitz G, Smoller BR, McNutt NS (1991) Benign cutaneous polyarteritis nodosa. Relationship to systemic polyarteritis nodosa and to hepatitis B infection. Arch Dermatol 127:1520–1523

    Article  CAS  PubMed  Google Scholar 

  100. David J, Ansell BM, Woo P (1993) Polyarteritis nodosa associated with streptococcus. Arch Dis Child 69:685–688

    Article  CAS  PubMed  Google Scholar 

  101. Stone MS, Olson RR, Weismann DN, Giller RH, Goeken JA (1993) Cutaneous vasculitis in the newborn of a mother with cutaneous polyarteritis nodosa. J Am Acad Dermatol 28:101–105

    Article  CAS  PubMed  Google Scholar 

  102. Boren RJ, Everett MA (1965) Cutaneous vasculitis in mother and child. Arch Dermatol 92:568–570

    Article  CAS  PubMed  Google Scholar 

  103. Borrie P (1972) Cutaneous polyarteritis nodosa. Br J Dermatol 87:87–95

    Article  CAS  PubMed  Google Scholar 

  104. Chen KR (1989) Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases. J Dermatol 16:429–442

    CAS  PubMed  Google Scholar 

  105. Dewar CL, Bellamy N (1992) Necrotizing mesenteric vasculitis after longstanding cutaneous polyarteritis nodosa. J Rheumatol 19:1308–1311

    CAS  PubMed  Google Scholar 

  106. Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, Furukawa F (2009) Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res 301:117–121

    Article  PubMed  Google Scholar 

  107. Siberry GK, Cohen BA, Johnson B (1994) Cutaneous polyarteritis nodosa. Reports of two cases in children and review of the literature. Arch Dermatol 130:884–889

    Article  CAS  PubMed  Google Scholar 

  108. Falcini F (2004) Vascular and connective tissue diseases in the paediatric world. Lupus 13:77–84

    Article  CAS  PubMed  Google Scholar 

  109. Fink CW (1991) The role of the streptococcus in poststreptococcal reactive arthritis and childhood polyarteritis nodosa. J Rheumatol Suppl 29:14–20

    CAS  PubMed  Google Scholar 

  110. Uziel Y, Silverman ED (1998) Intravenous immunoglobulin therapy in a child with cutaneous polyarteritis nodosa. Clin Exp Rheumatol 16:187–189

    CAS  PubMed  Google Scholar 

  111. Schartz NE, Alaoui S, Vignon-Pennamen MD, Cordoliani F, Fermand JP, Morel P, Rybojad M (2001) Successful treatment in two cases of steroid-dependent cutaneous polyarteritis nodosa with low-dose methotrexate. Dermatology 203:336–338

    Article  CAS  PubMed  Google Scholar 

  112. Maillard H, Szczesniak S, Martin L, Garot D, Machet MC, Machet L, Lorette G, Vaillant L (1999) Cutaneous periarteritis nodosa: diagnostic and therapeutic aspects of 9 cases. Ann Dermatol Venereol 126:125–129

    CAS  PubMed  Google Scholar 

  113. Calderon MJ, Landa N, Aguirre A, Diaz-Perez JL (1993) Successful treatment of cutaneous PAN with pentoxifylline. Br J Dermatol 128:706–708

    Article  CAS  PubMed  Google Scholar 

  114. Ginarte M, Pereiro M, Toribio J (1998) Cutaneous polyarteritis nodosa in a child. Pediatr Dermatol 15:103–107

    Article  CAS  PubMed  Google Scholar 

  115. Kawasaki T (1967) Acute febrile mucocutaneous syndrome in young children with unique digital desquamation. Jpn J Allergol 16:178–222

    CAS  Google Scholar 

  116. Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dillon MJ (2002) Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child 86:286–290

    Article  CAS  PubMed  Google Scholar 

  117. Burns JC, Glode MP (2004) Kawasaki syndrome. Lancet 364:533–544

    Article  PubMed  Google Scholar 

  118. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, Shulman ST, Bolger AF, Ferrieri P, Baltimore RS, Wilson WR, Baddour LM, Levison ME, Pallasch TJ, Falace DA, Taubert KA (2004) 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, American Heart Association. Pediatrics 114:1708–1733

    Article  PubMed  Google Scholar 

  119. Harnden A, Takahashi M, Burgner D (2009) Kawasaki disease. BMJ 338:b1514

    Article  PubMed  Google Scholar 

  120. Shulman ST, De Inocencio J, Hirsch R (1995) Kawasaki disease. Pediatr Clin North Am 42:1205–1222

    CAS  PubMed  Google Scholar 

  121. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197–1202

    Article  PubMed  Google Scholar 

  122. Tizard EJ (1999) Recognition and management of Kawasaki disease. Curr Pediatr 8:97–101

    Google Scholar 

  123. Yanagawa H, Nakamura Y, Yashiro M, Uehara R, Oki I, Kayaba K (2006) Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999–2002. Pediatr Int 48:356–361

    Article  PubMed  Google Scholar 

  124. Holman RC, Curns AT, Belay ED, Steiner CA, Schonberg LB (2003) Kawasaki syndrome hospitalizations in the United States, 1997 and 2000. Pediatrics 112:495–501

    Article  PubMed  Google Scholar 

  125. Harnden A, Alves B, Sheikh A (2002) Rising incidence of Kawasaki disease in England: analysis of hospital admission data. BMJ 324:1424–1425

    Article  PubMed  Google Scholar 

  126. Japan Kawasaki Disease Research Committee (2002) Diagnostic Guidelines for Kawasaki Disease. 5th Edition, Tokyo

  127. Council on Cardiovascular Disease in the Young, Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease; American Heart Association (2001) Diagnostic guidelines for Kawasaki Disease. Circulation 103:335–336

    Google Scholar 

  128. Dajani AS, Taubert KA, Gerber MA, Shulman ST, Ferrieri P, Freed M, Takahashi M, Bierman FZ, Karchmer AW, Wilson W (1993) Diagnosis and therapy of Kawasaki disease in children. Circulation 87:1776–1780

    CAS  PubMed  Google Scholar 

  129. Kato H, Takahashi K (2008) Kawasaki disease. In: Ball GV, Bridges SL (eds) Vasculitis, 2nd edn. Oxford University Press, Oxford, pp 373–389

    Google Scholar 

  130. Suzuki A, Kamiya T, Kuwahara N, Ono Y, Kohata T, Takahashi O, Kimura K, Takamiya M (1986) Coronary arterial lesions of Kawasaki disease: cardiac catheterization findings of 1100 cases. Pediatr Cardiol 7:3–9

    Article  CAS  PubMed  Google Scholar 

  131. Zulian F, Falcini F, Zancan L, Martini G, Secchieri S, Luzzatto C, Zacchello F (2003) Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr 142:731–735

    Article  PubMed  Google Scholar 

  132. Suddleson EA, Reid B, Woolley MM, Takahashi M (1987) Hydrops of the gallbladder associated with Kawasaki syndrome. J Pediatr Surg 22:956–959

    Article  CAS  PubMed  Google Scholar 

  133. Freeman AF, Crawford SE, Finn LS, Lopez-Andreu JA, Ferrando-Monleon S, Perez-Tamarit D, Cornwall ML, Shulman ST, Rowley AH (2003) Inflammatory pulmonary nodules in Kawasaki disease. Pediatr Pulmonol 36:102–106

    Article  PubMed  Google Scholar 

  134. Dengler LD, Capparelli EV, Bastian JF, Bradley DJ, Glode MP, Santa S, Newburger JW, Baker AL, Matsubara T, Burns JC (1998) Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J 17:478–481

    Article  CAS  PubMed  Google Scholar 

  135. Knott PD, Orloff LA, Harris JP, Novak RE, Burns JC, Kawasaki Disease Multicenter Hearing Loss Study Group (2001) Sensorineural hearing loss and Kawasaki disease: a prospective study. Am J Otolaryngol 22:343–348

    Article  CAS  PubMed  Google Scholar 

  136. Gong GW, McCrindle BW, Ching JC, Yeung RS (2006) Arthritis presenting during the acute phase of Kawasaki disease. J Pediatr 148:800–805

    Article  PubMed  Google Scholar 

  137. Melish ME, Hicks RM, Larson EJ (1976) Mucocutaneous lymph node syndrome in the United States. Am J Dis Child 130:599–607

    CAS  PubMed  Google Scholar 

  138. Ferriero DM, Wolfsdorf JI (1981) Hemolytic uremic syndrome associated with Kawasaki disease. Pediatrics 68:405–406

    CAS  PubMed  Google Scholar 

  139. Sasaguri Y, Kato H (1982) Regression of aneurysms in Kawasaki disease: a pathological study. J Pediatr 100:225–231

    Article  CAS  PubMed  Google Scholar 

  140. Salcedo JR, Greenberg L, Kapur S (1988) Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease). Clin Nephrol 29:47–51

    CAS  PubMed  Google Scholar 

  141. Mac Ardle BM, Chambers TL, Weller SD, Tribe CR (1983) Acute renal failure in Kawasaki disease. J R Soc Med. 76:615–616

    CAS  PubMed  Google Scholar 

  142. Nardi PM, Haller JO, Friedman AP, Slovis TL, Schaffer RM (1985) Renal manifestations of Kawasaki’s disease. Pediatr Radiol 15:116–118

    Article  CAS  PubMed  Google Scholar 

  143. Tizard EJ, Suzuki A, Levin M, Dillon MJ (1991) Clinical aspects of 100 patients with Kawasaki disease. Arch Dis Child 66:185–188

    Article  CAS  PubMed  Google Scholar 

  144. Veiga PA, Pieroni D, Baier W, Feld LG (1992) Association of Kawasaki disease and interstitial nephritis. Pediatr Nephrol 6:421–423

    Article  CAS  PubMed  Google Scholar 

  145. Lande MB, Gleeson JG, Sundel RP (1993) Kawasaki disease and acute renal failure. Pediatr Nephrol 7:593

    Article  CAS  PubMed  Google Scholar 

  146. Papadodima SA, Sakelliadis EI, Goutas ND, Vlachodimitropoulos DG, Spiliopoulou CA (2009) Atypical kawasaki disease presenting with symptoms from the genitourinary system: an autopsy report. J Trop Pediatr 55:55–57

    Article  PubMed  Google Scholar 

  147. Sevin C, Heidet L, Gagnadoux MF, Cheron G, Niaudet P (1993) Acute renal insufficiency in Kawasaki disease. Arch Fr Pediatr 50:505–507

    CAS  PubMed  Google Scholar 

  148. Tanaka H, Waga S, Tateyama T, Sugimoto K, Kakizaki Y, Yokoyama M (1999) Acute tubulointerstitial nephritis following intravenous immunoglobulin therapy in a male infant with minimal-change nephrotic syndrome. Tohoku J Exp Med 189:155–161

    Article  CAS  PubMed  Google Scholar 

  149. Kuniyuki S, Asada M (1997) An ulcerated lesion at the BCG vaccination site during the course of Kawasaki disease. J Am Acad Dermatol 37:303–304

    Article  CAS  PubMed  Google Scholar 

  150. Sireci G, Dieli F, Salerno A (2000) T cells recognize an immunodominant epitope of heat shock protein 65 in Kawasaki disease. Mol Med 6:581–590

    CAS  PubMed  Google Scholar 

  151. Wood LE, Tulloh RM (2009) Kawasaki disease in children. Heart 95:787–792

    Article  CAS  PubMed  Google Scholar 

  152. Zilberman MV, Goya G, Witt SA, Glascock B, Kimball TR (2003) Dobutamine stress echocardiography in the evaluation of young patients with Kawasaki disease. Pediatr Cardiol 24:338–343

    Article  CAS  PubMed  Google Scholar 

  153. Sato Y, Kato M, Inoue F, Fukui T, Imazeki T, Mitsui M, Matsumoto N, Takahashi M, Karasawa K, Ayusawa M, Kanamaru H, Harada K, Kanmatsuse K (2003) Detection of coronary artery aneurysms, stenoses and occlusions by multislice spiral computed tomography in adolescents with Kawasaki disease. Circ J 67:427–430

    Article  PubMed  Google Scholar 

  154. Greil GF, Stuber M, Botnar RM, Kissinger KV, Geva T, Newburger JW, Manning WJ, Powell AJ (2002) Coronary magnetic resonance angiography in adolescents and young adults with Kawasaki disease. Circulation 105:908–911

    Article  PubMed  Google Scholar 

  155. Brogan PA (2007) What’s new in the aetiopathogenesis of vasculitis? Pediatr Nephrol 22:1083–1094

    Article  PubMed  Google Scholar 

  156. Hsueh KC, Lin YJ, Chang JS, Wan L, Tsai YH, Tsai CH, Tsai FJ (2008) Influence of interleukin 18 promoter polymorphisms in susceptibility to Kawasaki disease in Taiwan. J Rheumatol 35:1408–1413

    CAS  PubMed  Google Scholar 

  157. Burgner D, Davila S, Breunis WB, Ng SB, Li Y, Bonnard C, Ling L, Wright VJ, Thalamuthu A, Odam M, Shimizu C, Burns JC, Levin M, Kuijpers TW, Hibberd ML (2009) A genome-wide association study identifies novel and functionally related susceptibility Loci for Kawasaki disease. PLoS Genet 5:e1000319

    Article  CAS  PubMed  Google Scholar 

  158. Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, Nakamura Y, Yanagawa H, Wakui K, Fukushima Y, Kishi F, Hamamoto K, Terai M, Sato Y, Ouchi K, Saji T, Nariai A, Kaburagi Y, Yoshikawa T, Suzuki K, Tanaka T, Nagai T, Cho H, Fujino A, Sekine A, Nakamichi R, Tsunoda T, Kawasaki T, Nakamura Y, Hata A (2008) ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nat Genet 40:35–42

    Article  CAS  PubMed  Google Scholar 

  159. Matsubara K, Fukaya T, Miwa K, Shibayama N, Nigami H, Harigaya H, Nozaki H, Hirata T, Baba K, Suzuki T, Ishiguro A (2006) Development of serum IgM antibodies against superantigens of Staphylococcus aureus and Streptococcus pyogenes in Kawasaki disease. Clin Exp Immunol 143:427–434

    Article  CAS  PubMed  Google Scholar 

  160. Rowley AH, Shulman ST, Spike BT, Mask CA, Baker SC (2001) Oligoclonal IgA response in the vascular wall in acute Kawasaki disease. J Immunol 166:1334–1343

    CAS  PubMed  Google Scholar 

  161. Durongpisitkul K, Gururaj VJ, Park JM, Martin CF (1995) The prevention of coronary artery aneurysms in Kawasaki disease: a meta-analysis on the effects of aspirin and immunoglobulin treatment. Pediatrics 96:1057–1061

    CAS  PubMed  Google Scholar 

  162. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, Atz AM, Li JS, Takahashi M, Baker AL, Colan SD, Mitchell PD, Klein GL, Sundel RP (2007) Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med 356:663–675

    Article  CAS  PubMed  Google Scholar 

  163. Kijima Y, Kamiya T, Suzuki A, Hirose O, Manabe H (1982) A trial procedure to prevent aneurysm formation of the coronary arteries by steroid pulse therapy in Kawasaki disease. Jpn Circ J 46:1239–1242

    CAS  PubMed  Google Scholar 

  164. Sundel RP, Baker AL, Fulton DR, Newburger JW (2003) Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial. J Pediatr 142:611–616

    Article  CAS  PubMed  Google Scholar 

  165. Inoue Y, Okada Y, Shinohara M, Kobayashi T, Kobayashi T, Tomomasa T, Takeuchi K, Morikawa A (2006) A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr 149:336–341

    Article  CAS  PubMed  Google Scholar 

  166. Shinohara M, Sone K, Tomomasa T, Morikawa A (1999) Corticosteroids in the treatment of the acute phase of Kawasaki disease. J Pediatr 135:465–469

    Article  CAS  PubMed  Google Scholar 

  167. Dale RC, Saleem MA, Daw S, Dillon MJ (2000) Treatment of severe complicated Kawasaki disease with oral prednisolone and aspirin. J Pediatr 137:723–726

    Article  CAS  PubMed  Google Scholar 

  168. Furukawa T, Kishiro M, Akimoto K, Nagata S, Shimizu T, Yamashiro Y (2008) Effects of steroid pulse therapy on immunoglobulin-resistant Kawasaki disease. Arch Dis Child 93:142–146

    Article  CAS  PubMed  Google Scholar 

  169. Okada K, Hara J, Maki K, Matsuzaki K, Matsuoka T, Yamamoto T, Nishigaki T, Kuotobi S, Sano T, Osaka Kawasaki Disease Study Group (2009) Pulse methylprednisolone with gammaglobulin as an initial treatment for acute Kawasaki disease. Eur J Pediatr 168:181–185

    Article  CAS  PubMed  Google Scholar 

  170. Brogan RJ, Eleftheriou D, Gnanapragasam J, Klein NJ, Brogan PA (2009) Infliximab for the treatment of intravenous immunoglobulin resistant Kawasaki disease complicated by coronary artery aneurysms: a case report. Pediatr Rheumatol Online J 7:3

    Article  PubMed  Google Scholar 

  171. Burns JC, Best BM, Mejias A, Mahony L, Fixler DE, Jafri HS, Melish ME, Jackson MA, Asmar BI, Lang DJ, Connor JD, Capparelli EV, Keen ML, Mamun K, Keenan GF, Ramilo O (2008) Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease. J Pediatr 153:833–838

    Article  CAS  PubMed  Google Scholar 

  172. Nakamura Y, Aso E, Yashiro M, Uehara R, Watanabe M, Oki I, Yanagawa H (2008) Mortality among persons with a history of Kawasaki disease in Japan: mortality among males with cardiac sequelae is significantly higher than that of the general population. Circ J 72:134–138

    Article  PubMed  Google Scholar 

  173. Phillip R, Luqmani R (2008) Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol 26:S94–S104

    CAS  PubMed  Google Scholar 

  174. Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno Y, Kazue T, Eto G, Yamakawa R (1996) Long-term consequences of Kawasaki disease. A 10− to 21-year follow-up study of 594 patients. Circulation 94:1379–1385

    CAS  PubMed  Google Scholar 

  175. Tatara K, Kusakawa S (1987) Long-term prognosis of giant coronary aneurysm in Kawasaki disease: an angiographic study. J Pediatr 111:705–710

    Article  CAS  PubMed  Google Scholar 

  176. Dhillon R, Clarkson P, Donald AE, Powe AJ, Nash M, Novelli V, Dillon MJ, Deanfield JE (1996) Endothelial dysfunction late after Kawasaki disease. Circulation 94:2103–2106

    CAS  PubMed  Google Scholar 

  177. Niboshi A, Hamaoka K, Sakata K, Yamaguchi N (2008) Endothelial dysfunction in adult patients with a history of Kawasaki disease. Eur J Pediatr 167:189–196

    Article  CAS  PubMed  Google Scholar 

  178. Mitani Y, Okuda Y, Shimpo H, Uchida F, Hamanaka K, Aoki K, Sakurai M (1997) Impaired endothelial function in epicardial coronary arteries after Kawasaki disease. Circulation 96:454–461

    CAS  PubMed  Google Scholar 

  179. Cheung YF, Wong SJ, Ho MH (2007) Relationship between carotid intima-media thickness and arterial stiffness in children after Kawasaki disease. Arch Dis Child 92:43–47

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Nephrourology Unit, UCL Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK

    Michael J. Dillon

  2. Department of Rheumatology, UCL Institute of Child Health, London, UK

    Despina Eleftheriou & Paul A. Brogan

Authors
  1. Michael J. Dillon
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  2. Despina Eleftheriou
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Additional information

Answers:

1. d

2. b

3. c

4. a

5. c

Multiple-choice questions: (Answers appear following the reference list)

Multiple-choice questions: (Answers appear following the reference list)

  1. 1.

    Which of the following is true in relation to childhood PAN?

  2. a.

    Crescentic glomerulonephritis is a well-recognised complication

  3. b.

    There is often a discrepancy between the ESR and the CRP results

  4. c.

    Cytoplasmic ANCA is usually detectable

  5. d.

    Livido reticularis is a characteristic feature

  6. 2.

    In childhood PAN, which of the following statements is correct?

  7. a.

    Most cases have an infective aetiology

  8. b.

    Renal and hepatic angiography is a valuable investigative tool

  9. c.

    The aorta is frequently affected

  10. d.

    Cyclophosphamide is contraindicated because of side effects

  11. 3.

    In relation to cutaneous childhood PAN, which of the following is true?

  12. a.

    Pyoderma gangrenosum is a severe manifestation

  13. b.

    Leukocytoclastic vasculitis is a characteristic histological finding

  14. c.

    The condition rarely progresses to systemic PAN

  15. d.

    It is the commonest cause of vasculitis involving the skin in children

  16. 4.

    Regarding KD, which of the following is correct?

  17. a.

    IV-Ig can be effective if administered later than 10 days after disease onset

  18. b.

    Only coronary arteries are affected

  19. c.

    Acute glomerulonephritis is the usual renal manifestation

  20. d.

    The disease is unlikely unless all criteria are met

  21. 5.

    For KD, which of the following is false?

  22. a.

    There is evidence for genetically determined susceptibility to the disease

  23. b.

    Long-term endothelial dysfunction has been demonstrated after the acute phase

  24. c.

    Female patients with cardiac sequelae have a higher mortality rate than male patients

  25. d.

    There are data supporting an aetiological role for superantigens

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Dillon, M.J., Eleftheriou, D. & Brogan, P.A. Medium-size-vessel vasculitis. Pediatr Nephrol 25, 1641–1652 (2010). https://doi.org/10.1007/s00467-009-1336-1

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  • DOI: https://doi.org/10.1007/s00467-009-1336-1

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