Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Pediatric Nephrology
Published in: Pediatric Nephrology 6/2010

01-06-2010 | Educational Review

Small vessel vasculitis

Authors: Paul Brogan, Despina Eleftheriou, Michael Dillon

Published in: Pediatric Nephrology | Issue 6/2010

Login to get access

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.
Appendix
Available only for authorised users
Literature
1.
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
2.
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
3.
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
4.
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
5.
6.
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
7.
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
8.
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
9.
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.
41.
42.
Harper L, Savage CO (2000) Pathogenesis of ANCA-associated systemic vasculitis. J Pathol 190:349–359CrossRefPubMed
43.
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.
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
Metadata
Title
Small vessel vasculitis
Authors
Paul Brogan
Despina Eleftheriou
Michael Dillon
Publication date
01-06-2010
Publisher
Springer-Verlag
Published in
Pediatric Nephrology / Issue 6/2010
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-009-1317-4

Other articles of this Issue 6/2010

Pediatric Nephrology 6/2010 Go to the issue

Brief Report

Cryptogenic organizing pneumonia after rituximab therapy for presumed post-kidney transplant lymphoproliferative disease

Original Article

Predictors of outcome in Henoch–Schönlein nephritis

Brief Report

Two families with compound heterozygosity for adenine phosphoribosyltransferase deficiency

Original Article

Rituximab efficiency in children with steroid-dependent nephrotic syndrome

Review

Enzymatic disease of the podocyte

Brief Report

Glomerular basement membrane lipidosis in Alagille syndrome