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Vasculitis in children: Management overview

David Cabral, MBBS, FRCPC
Kimberly Morishita, MD, MHSc, FRCPC
Section Editor
Robert Sundel, MD
Deputy Editor
Elizabeth TePas, MD, MS


Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely. The various vasculitic syndromes are defined by both the type of pathologic inflammation and the type and location of the vessels and this, in turn, determines which specific organs are affected [1,2].

The first priorities in management of a child with vasculitis are prompt recognition and timely treatment since many of the vasculitides can be severe and life-threatening conditions if not appropriately managed. Specific treatments are determined by the type of vasculitis, the severity of the inflammation, and the particular organ systems affected.

A general overview of the management of vasculitis in children is reviewed here. The classification and the approach to evaluating childhood vasculitis are discussed separately. (See "Vasculitis in children: Classification and incidence" and "Vasculitis in children: Evaluation".)


The following principles are important to consider when managing a child with vasculitis:

Differentiating vasculitis from other diseases that can either mimic vasculitis or cause secondary vasculitis

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Literature review current through: Nov 2017. | This topic last updated: Jul 10, 2017.
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  1. Cassidy JT, Petty RE. Vasculitis and its classification. In: Textbook of pediatric rheumatology, 5th ed, Cassidy JT, Petty RE (Eds), Elsevier Saunders, Philadelphia 2005. p.492.
  2. Dedeoglu F, Sundel RP. Vasculitis in children. Rheum Dis Clin North Am 2007; 33:555.
  3. Hunder G. Vasculitis: diagnosis and therapy. Am J Med 1996; 100:37S.
  4. Roane DW, Griger DR. An approach to diagnosis and initial management of systemic vasculitis. Am Fam Physician 1999; 60:1421.
  5. Langford CA. Management of systemic vasculitis. Best Pract Res Clin Rheumatol 2001; 15:281.
  6. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
  7. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337:1512.
  8. Watts RA, Suppiah R, Merkel PA, Luqmani R. Systemic vasculitis--is it time to reclassify? Rheumatology (Oxford) 2011; 50:643.
  9. Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol 2006; 24:414.
  10. Weiss PF, Klink AJ, Localio R, et al. Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schönlein purpura. Pediatrics 2010; 126:674.
  11. Zaffanello M, Fanos V. Treatment-based literature of Henoch-Schönlein purpura nephritis in childhood. Pediatr Nephrol 2009; 24:1901.
  12. Tarshish P, Bernstein J, Edelmann CM Jr. Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 2004; 19:51.
  13. Kang Z, Li Z, Duan C, et al. Mycophenolate mofetil therapy for steroid-resistant IgA nephropathy with the nephrotic syndrome in children. Pediatr Nephrol 2015; 30:1121.
  14. Fenoglio R, Naretto C, Basolo B, et al. Rituximab therapy for IgA-vasculitis with nephritis: a case series and review of the literature. Immunol Res 2017; 65:186.
  15. Eleftheriou D, Melo M, Marks SD, et al. Biologic therapy in primary systemic vasculitis of the young. Rheumatology (Oxford) 2009; 48:978.
  16. Wilkinson NM, Erendzhinova E, Zeft A, Cabral DA. Infliximab as rescue therapy in three cases of paediatric Wegener's granulomatosis. Rheumatology (Oxford) 2006; 45:1047.
  17. Stern S, Clemente G, Reiff A, et al. Treatment of Pediatric Takayasu arteritis with infliximab and cyclophosphamide: experience from an American-Brazilian cohort study. J Clin Rheumatol 2014; 20:183.
  18. Szugye HS, Zeft AS, Spalding SJ. Takayasu Arteritis in the pediatric population: a contemporary United States-based single center cohort. Pediatr Rheumatol Online J 2014; 12:21.
  19. Goel R, Kumar TS, Danda D, et al. Childhood-onset Takayasu arteritis -- experience from a tertiary care center in South India. J Rheumatol 2014; 41:1183.
  20. Cañas CA, Cañas F, Izquierdo JH, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol 2014; 20:125.
  21. Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum 1994; 37:578.
  22. 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.
  23. Allen NB, Bressler PB. Diagnosis and treatment of the systemic and cutaneous necrotizing vasculitis syndromes. Med Clin North Am 1997; 81:243.
  24. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003; 349:36.
  25. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 2009; 150:670.
  26. Heijl C, Harper L, Flossmann O, et al. Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials. Ann Rheum Dis 2011; 70:1415.
  27. Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363:211.
  28. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363:221.
  29. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 2014; 371:1771.
  30. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68:310.
  31. Geetha D, Kallenberg C, Stone JH, et al. Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol 2015; 28:17.
  32. De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005; 52:2461.
  33. Jayne D. How to induce remission in primary systemic vasculitis. Best Pract Res Clin Rheumatol 2005; 19:293.
  34. Langford CA, Sneller MC, Hoffman GS. Methotrexate use in systemic vasculitis. Rheum Dis Clin North Am 1997; 23:841.
  35. Langford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum 2000; 43:1836.
  36. Stone JH, Tun W, Hellman DB. Treatment of non-life threatening Wegener's granulomatosis with methotrexate and daily prednisone as the initial therapy of choice. J Rheumatol 1999; 26:1134.
  37. Langford CA, Talar-Williams C, Barron KS, Sneller MC. A staged approach to the treatment of Wegener's granulomatosis: induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. Arthritis Rheum 1999; 42:2666.
  38. Langford CA, Talar-Williams C, Barron KS, Sneller MC. Use of a cyclophosphamide-induction methotrexate-maintenance regimen for the treatment of Wegener's granulomatosis: extended follow-up and rate of relapse. Am J Med 2003; 114:463.
  39. Singer O, McCune WJ. Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. Curr Opin Rheumatol 2017; 29:248.
  40. Guerry MJ, Brogan P, Bruce IN, et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford) 2012; 51:634.
  41. Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007; 18:2180.
  42. Szpirt WM, Heaf JG, Petersen J. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial. Nephrol Dial Transplant 2011; 26:206.
  43. Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials 2013; 14:73.
  44. Tann OR, Tulloh RM, Hamilton MC. Takayasu's disease: a review. Cardiol Young 2008; 18:250.
  45. Falcini F, Generini S, Pignone A, et al. Are Angiotensin Converting Enzyme and von Willebrand factor circulating levels useful surrogate parameters to monitor disease activity in Kawasaki disease? Endothelium 1999; 6:209.
  46. Fazzini F, Peri G, Doni A, et al. PTX3 in small-vessel vasculitides: an independent indicator of disease activity produced at sites of inflammation. Arthritis Rheum 2001; 44:2841.
  47. Dolezalova P, Price-Kuehne FE, Özen S, et al. Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). Ann Rheum Dis 2013; 72:1628.
  48. Moorthy AV, Chesney RW, Segar WE, Groshong T. Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy. J Pediatr 1977; 91:616.
  49. WALTON EW. Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J 1958; 2:265.
  50. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol 2008; 26:S94.
  51. Morishita KA, Moorthy LN, Lubieniecka JM, et al. Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol 2017; 69:1470.
  52. Rottem M, Fauci AS, Hallahan CW, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr 1993; 122:26.
  53. Akikusa JD, Schneider R, Harvey EA, et al. Clinical features and outcome of pediatric Wegener's granulomatosis. Arthritis Rheum 2007; 57:837.
  54. Iudici M, Puéchal X, Pagnoux C, et al. Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry. Arthritis Rheumatol 2015; 67:1959.
  55. Exley AR, Carruthers DM, Luqmani RA, et al. Damage occurs early in systemic vasculitis and is an index of outcome. QJM 1997; 90:391.