nach oben

Pediatric Nephrology

Erschienen in:

01.06.2010 | Educational Review

Small vessel vasculitis

verfasst von: Paul Brogan, Despina Eleftheriou, Michael Dillon

Erschienen in: Pediatric Nephrology | Ausgabe 6/2010

Einloggen, um Zugang zu erhalten

Abstract

The pediatric small vessel vasculitides reviewed in this article are Henoch–Schönlein purpura (HSP) and the anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV). The new classification criteria for HSP and Wegener’s granulomatosis are now validated and will facilitate the conduct of future epidemiological studies and clinical trials. The clinical manifestations of small vessel vasculitis in children are described, and current therapies discussed. There is a lack of good clinical trial data on which to base therapy for HSP. Similarly, data based on randomized controlled trials (RCTs) for pediatric AAV are lacking, although children with AAV are for the first time now included in a RCT of mycophenolate mofetil versus cyclophosphamide. Significant challenges remain in the field of pediatric small vessel vasculitis, including the development of validated disease outcome measures and biomarkers to be used in clinical trials. Lastly, long-term outcome data are lacking in survivors of pediatric small vessel vasculitis.

Nur mit Berechtigung zugänglich

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192CrossRefPubMed

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

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 65:936–941CrossRefPubMed

Ozen S, Pistorio A, Iusan M, Bakkaloglu A, Herlin T, Brik R, Uziel S, Stabile A, Cantarini L, Norambuena X, Berkun Y, Olivieri AN, Djeddi D, Nuno L, Chasnyk V, Pruunsild C, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2009) The EULAR/ PRINTO/PRES criteria for Henoch-Schönlein purpura. Ann Rheum Dis 68[Suppl 3]:712

Gedalia A (2004) Henoch-Schönlein purpura. Curr Rheumatol Rep 6:195–202CrossRefPubMed

Dolezalova P, Telekesova P, Nemcova D, Hoza J (2004) Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. J Rheumatol 31:2295–2299PubMed

Aalberse J, Dolman K, Ramnath G, Pereira RR, Davin JC (2007) Henoch Schönlein purpura in children: an epidemiological study among Dutch paediatricians on incidence and diagnostic criteria. Ann Rheum Dis 66:1648–1650CrossRefPubMed

Yang YH, Hung CF, Hsu CR, Wang LC, Chuang YH, Lin YT, Chiang BL (2005) A nationwide survey on epidemiological characteristics of childhood Henoch-Schönlein purpura in Taiwan. Rheumatology (Oxford) 44:618–622CrossRef

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

10.

Amoli MM, Thomson W, Hajeer AH, Calvino MC, Garcia-Porrua C, Ollier WE, Gonzalez-Gay MA (2002) Interleukin 1 receptor antagonist gene polymorphism is associated with severe renal involvement and renal sequelae in Henoch-Schönlein purpura. J Rheumatol 29:1404–1407PubMed

11.

Gershoni-Baruch R, Broza Y, Brik R (2003) Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schönlein purpura. J Pediatr 143:658–661CrossRefPubMed

12.

Cheung KM, Mok F, Lam P, Chan KH (2001) Pancreatitis associated with Henoch-Schönlein purpura. J Paediatr Child Health 37:311–313CrossRefPubMed

13.

Siomou E, Serbis A, Salakos C, Papadopoulou F, Stefanidis CJ, Siamopoulou A (2008) Masked severe stenosing ureteritis: a rare complication of Henoch-Schönlein purpura. Pediatr Nephrol 23:821–825CrossRefPubMed

14.

Webb NJA, Brogan PA, Baildam EM (2003) Renal manifestations of systemic disorders. In: Webb N, Postlethwaite R (eds) Clinical paediatric nephrology. Oxford University Press, Oxford, pp 381–403

15.

Koskimies O, Rapola J, Savilahti E, Vilska J (1974) Renal involvement in Henoch Schönlein purpura. Acta Pediatr Scand 63:357–363CrossRef

16.

Stewart M, Savage JM, Bell B, McCord B (1988) Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population. Eur J Pediatr 147:113–115CrossRefPubMed

17.

Lin JJ, Stewart CL, Kaskel FJ, Fine RN (1993) IgG and IgA classes of anti-neutrophil cytoplasmic autoantibodies in a 13-year-old girl with recurrent Henoch-Schönlein purpura. Pediatr Nephrol 7:143–146CrossRefPubMed

18.

Lau KK, Wyatt RJ, Moldoveanu Z, Tomana M, Julian BA, Hogg RJ, Lee JY, Huang WQ, Mestecky J, Novak J (2007) Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schönlein purpura. Pediatr Nephrol 22:2067–2072CrossRefPubMed

19.

Shin JI, Kim JH, Lee JS (2008) The diagnostic value of IgA deposition in Henoch-Schönlein purpura. Pediatr Dermatol 25:140–141CrossRefPubMed

20.

Gunasekaran TS, Berman J, Gonzalez M (2000) Duodenojejunitis: is it idiopathic or is it Henoch-Schönlein purpura without the purpura? J Pediatr Gastroenterol Nutr 30:22–28CrossRefPubMed

21.

Kamitsuji H, Tani K, Yasui M, Taniguchi A, Taira K, Tsukada S, Iida Y, Kanki H, Fukui H (1987) Activity of blood coagulation factor XIII as a prognostic indicator in patients with Henoch-Schönlein purpura. Efficacy of factor XIII substitution. Eur J Pediatr 146:519–523CrossRefPubMed

22.

Kawasaki K, Komura H, Nakahara Y, Shiraishi M, Higashida M, Ouchi K (2006) Factor XIII in Henoch-Schönlein purpura with isolated gastrointestinal symptoms. Pediatr Int 48:413–415CrossRefPubMed

23.

Rees L, Webb NJA, Brogan PA (2007) Vasculitis. In: Rees L, Webb NJA, Brogan PA (eds) Paediatric nephrology (Oxford Handbook). Oxford University Press, Oxford, pp 310–313

24.

Carlson JA, Chen KR (2006) Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndromes. Am J Dermatopathol 28:486–506CrossRefPubMed

25.

Flatau E, Kohn D, Schiller D, Lurie M, Levy E (1982) Schönlein-Henoch syndrome in patients with familial Mediterranean fever. Arthritis Rheum 25:42–47CrossRefPubMed

26.

Ozdogan H, Arisoy N, Kasapcapur O, Sever L, Caliskan S, Tuzuner N, Mat C, Yazici H (1997) Vasculitis in familial Mediterranean fever. J Rheumatol 24:323–327PubMed

27.

Zaffanello M, Fanos V (2009) Treatment-based literature of Henoch-Schönlein purpura nephritis in childhood. Pediatr Nephrol 24:1901–1911

28.

Huber AM, King J, McLaine P, Klassen T, Pothos M (2004) A randomized, placebo-controlled trial of prednisone in early Henoch-Schönlein purpura [ISRCTN85109383]. BMC Med 2:7CrossRefPubMed

29.

Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, Ormala T, Turtinen J, Nuutinen M (2006) Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr 149:241–247CrossRefPubMed

30.

Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM (2009) Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review. Arch Dis Child 94:132–137CrossRefPubMed

31.

Tarshish P, Bernstein J, Edelmann CM Jr (2004) Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19:51–56CrossRefPubMed

32.

Shenoy M, Ognjanovic MV, Coulthard MG (2007) Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol 22:1167–1171CrossRefPubMed

33.

Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243CrossRefPubMed

34.

Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 339:280–282CrossRefPubMed

35.

Narchi H (2005) 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 90:916–920CrossRefPubMed

36.

Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 360:666–670CrossRefPubMed

37.

Algoet C, Proesmans W (2003) Renal biopsy 2–9 years after Henoch Schönlein purpura. Pediat Nephrol 18:471–473PubMed

38.

Meulders Q, Pirson Y, Cosyns JP, Squifflet JP, van Ypersele DS (1994) Course of Henoch-Schönlein nephritis after renal transplantation. Report on ten patients and review of the literature. Transplantation 58:1179–1186CrossRefPubMed

39.

Hasegawa A, Kawamura T, Ito H, Hasegawa O, Ogawa O, Honda M, Ohara T, Hajikano H (1989) Fate of renal grafts with recurrent Henoch-Schönlein purpura nephritis in children. Transplant Proc 21:2130–2133PubMed

40.

Radice A, Sinico RA (2005) Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity 38:93–103CrossRefPubMed

41.

Zwerina J, Eger G, Englbrecht M, Manger B, Schett G (2008) Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum. doi:10.1016/j.semarthrit.2008.05.004 PubMed

42.

Harper L, Savage CO (2000) Pathogenesis of ANCA-associated systemic vasculitis. J Pathol 190:349–359CrossRefPubMed

43.

Dillon M, Eleftheriou D, Brogan P (2009) Medium-size vessel vasculitis. Pediatr Nephrol. doi:10.1007/s00467-009-1336-1

44.

Jardim HM, Leake J, Risdon RA, Barratt TM, Dillon MJ (1992) Crescentic glomerulonephritis in children. Pediatr Nephrol 6:231–235CrossRefPubMed

45.

Lindsley CB (2001) Granulomatous vasculitis, giant cell arteritis, and sarcoidosis. In: Cassidy JT, Petty RE (eds) Textbook of pediatric rheumatology. WB Saunders, Philadelphia, pp 604–627

46.

Belostotsky VM, Shah V, Dillon MJ (2002) Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol 17:754–761CrossRefPubMed

47.

Akikusa JD, Schneider R, Harvey EA, Hebert D, Thorner PS, Laxer RM, Silverman ED (2007) Clinical features and outcome of pediatric Wegener’s granulomatosis. Arthritis Rheum 57:837–844CrossRefPubMed

48.

Xiao H, Heeringa P, Hu P, Liu Z, Zhao M, Aratani Y, Maeda N, Falk RJ, Jennette JC (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 10:955–963

49.

Bansal PJ, Tobin MC (2004) Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement. Ann Allergy Asthma Immunol 93:398–401CrossRefPubMed

50.

Schlieben DJ, Korbet SM, Kimura RE, Schwartz MM, Lewis EJ (2005) Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis 45:758–761CrossRefPubMed

51.

Finkielman JD, Merkel PA, Schroeder D, Hoffman GS, Spiera R, St Clair EW, Davis JC Jr, McCune WJ, Lears AK, Ytterberg SR, Hummel AM, Viss MA, Peikert T, Stone JH, Specks U, WGET Research Group (2007) Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med 147:611–619PubMed

52.

Jayne D (2009) Review article: Progress of treatment in ANCA-associated vasculitis. Nephrology 14:42–48CrossRefPubMed

53.

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

54.

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

55.

de Groot K, Adu D, Savage CO (2001) The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 16:2018–2027CrossRefPubMed

56.

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

57.

Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, Ekstrand A, Gaskin G, Gregorini G, de Groot K, Gross W, Hagen EC, Mirapeix E, Pettersson E, Siegert C, Sinico A, Tesar V, Westman K, Pusey C, European Vasculitis Study Group (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349:36–44CrossRefPubMed

58.

Stassen PM, Derks RP, Kallenberg CG, Stegeman CA (2008) Venous thromboembolism in ANCA-associated vasculitis-incidence and risk factors. Rheumatology (Oxford) 47:530–534CrossRef

59.

de Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DR (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 52:2461–2469CrossRefPubMed

60.

Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 335:16–20CrossRef

61.

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

62.

Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH (2005) A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 20:2725–2732CrossRefPubMed

63.

Smith KG, Jones RB, Burns SM, Jayne DR (2006) Long-term comparison of rituximab treatment for refractory systemic lupus erythematosus and vasculitis: Remission, relapse, and re-treatment. Arthritis Rheum 54:2970–2982CrossRefPubMed

64.

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 (Oxford) 48:978–986CrossRef

65.

Hoffman GS, Thomas-Golbanov CK, Chan J, Akst LM, Eliachar I (2003) Treatment of subglottic stenosis, due to Wegener’s granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol 30:1017–1021PubMed

66.

White JB, Shah RK (2009) Wegener’s granulomatosis of the pediatric airway: a case demonstrating a conservative management approach. Am J Otolaryngol 30:212–215CrossRefPubMed

67.

Walton EW (1958) Giant-cell granuloma of the respiratory tract (Wegener’s granulomatosis). Br Med J 2:265–270CrossRefPubMed

68.

Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, Garcia-Consuegra J, Dolezalova P, Dressler F, Duzova A, Ferriani VP, Hilário MO, Ibáñez-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 145:517–522CrossRefPubMed

69.

Hattori M, Kurayama H, Koitabashi Y (2001) Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 12:1493–1500PubMed

70.

Peco-Antic A, Bonaci-Nikolic B, Basta-Jovanovic G, Kostic M, Markovic-Lipkovski J, Nikolic M, Spasojevic B (2006) Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol 21:46–53CrossRefPubMed

Titel: Small vessel vasculitis
verfasst von: Paul Brogan
Despina Eleftheriou
Michael Dillon
Publikationsdatum: 01.06.2010
Verlag: Springer-Verlag
Erschienen in: Pediatric Nephrology / Ausgabe 6/2010
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-009-1317-4

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Small vessel vasculitis

Abstract

Neu im Fachgebiet Pädiatrie

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Pädiatrie

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 6/2010

Levels of urinary transforming growth factor β-1 in children with D+ hemolytic uremic syndrome

Molecular anatomy of the kidney: what have we learned from gene expression and functional genomics?

IgG subclasses and complement pathway in segmental and global membranous nephropathy

Chronic treatment with lisinopril decreases proliferative and apoptotic pathways in autosomal recessive polycystic kidney disease

Imaging in the evaluation of renovascular disease

HNF1B alterations associated with congenital anomalies of the kidney and urinary tract

Neu im Fachgebiet Pädiatrie

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

Update Pädiatrie