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Renal Vasculitis in Childhood

  • Renal (R Parekh, Section Editor)
  • Published:
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Abstract

Purpose of Review

Renal vasculitis in children, while rare, can be life threatening and associated with significant morbidity and mortality. Although sometimes renal limited, more often, the kidney is affected as part of a more systemic disorder. While there are increasing numbers of pediatric case series, management decisions are derived from randomized controlled trials conducted in adults. The aim of this review is to summarize the recent literature on the pathogenesis, management, and outcome of small-, medium-, and large-vessel renal vasculitis in the pediatric population.

Recent Findings

Our understanding of the pathogenesis of these conditions has grown allowing more targeted therapies, such as rituximab or potentially complement pathway inhibitors in ANCA vasculitis and biologicals in polyarteritis nodosa and Takayasu’s arteritis. There is a new and validated histopathological classification schema that predicts renal outcome in pediatric ANCA vasculitis.

Summary

The results of key adult studies on optimal duration of immunosuppression maintenance are keenly awaited.

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References

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  1. Maritati F, Iannuzzella F, Pavia MP, Pasquali S, Vaglio A. Kidney involvement in medium- and large-vessel vasculitis. J Nephrol. 2016; doi:10.1007/s40620-016-0303-8.

    PubMed  Google Scholar 

  2. • Ozen SPA, Iusan S, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood poyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum dis. 2010;69:798–806. Pediatric-specific diagnostic criteria for systemic vasculitides.

    Article  PubMed  Google Scholar 

  3. • Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11. doi:10.1002/art.37715. Updated classification system for the systemic vasculitides.

    Article  CAS  PubMed  Google Scholar 

  4. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–8.

    CAS  PubMed  Google Scholar 

  5. • Dolezalova P, Price-Kuehne FE, Ozen S, Benseler SM, Cabral DA, Anton J et al. Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). Annals of the rheumatic diseases. 2013;72(10):1628–1633. doi: 10.1136/annrheumdis-2012-202111 This study validates an objective clinical tool for measuring disease activity in systemic vasculitis, to be used in future.

  6. Sacri AS, Chambaraud T, Ranchin B, Florkin B, See H, Decramer S, et al. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2015;30(Suppl 1):i104–12. doi:10.1093/ndt/gfv011.

    Google Scholar 

  7. Grisaru S, Yuen GWH, Miettunen PM, Hamiwka LA. Incidence of Wegener’s granulomatosis in children. J Rheumatol. 2009;37(2):440–2. doi:10.3899/jrheum.090688.

    Article  PubMed  Google Scholar 

  8. • Cabral DA, Canter DL, Muscal E, Nanda K, Wahezi DM, Spalding SJ, et al. Comparing presenting clinical features in 48 children with microscopic polyangiitis to 183 children who have granulomatosis with polyangiitis (Wegener’s): an ARChiVe cohort study. Arthritis & Rheumatology. 2016;68(10):2514–26. doi:10.1002/art.39729. A large registry study which describes the clinical features of GPA versus MPA in children.

    Article  CAS  Google Scholar 

  9. Schonermarck U, Csernok E, Gross WL. Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis: challenges and solutions 2014. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2015;30(Suppl 1):i46–52. doi:10.1093/ndt/gfu398.

    Google Scholar 

  10. Cabral DA, Uribe AG, Benseler S, O'Neil KM, Hashkes PJ, Higgins G, et al. Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum. 2009;60(11):3413–24. doi:10.1002/art.24876.

    Article  PubMed  Google Scholar 

  11. Siomou E, Tramma D, Bowen C, Milford DV. ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features-outcome. Pediatr Nephrol. 2012;27(10):1911–20. doi:10.1007/s00467-012-2198-5.

    Article  PubMed  Google Scholar 

  12. Sable-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern med. 2005;143(9):632–8.

    Article  PubMed  Google Scholar 

  13. Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis. Autoimmun rev. 2013;12(4):487–95. doi:10.1016/j.autrev.2012.08.008.

    Article  CAS  PubMed  Google Scholar 

  14. Lyons PA, Rayner TF, Trivedi S, Holle JU, Watts RA, Jayne DR, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J med. 2012;367(3):214–23. doi:10.1056/NEJMoa1108735.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, et al. Histopathologic classification of ANCA-associated glomerulonephritis. Journal of the American Society of Nephrology : JASN. 2010;21(10):1628–36. doi:10.1681/ASN.2010050477.

    Article  PubMed  Google Scholar 

  16. Bajema IM, Hagen EC, Ferrario F, de Heer E, Bruijn JA. Immunopathological aspects of systemic vasculitis. Springer Semin Immunopathol. 2001;23(3):253–65.

    Article  CAS  PubMed  Google Scholar 

  17. Jennette JC, Falk RJ. Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat rev Rheumatol. 2014;10(8):463–73. doi:10.1038/nrrheum.2014.103.

    Article  CAS  PubMed  Google Scholar 

  18. Sanders JS, Abdulahad WH, Stegeman CA, Kallenberg CG. Pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis and potential targets for biologic treatment. Nephron Clinical Practice. 2014;128(3–4):216–23. doi:10.1159/000368570.

    Article  CAS  PubMed  Google Scholar 

  19. Jennette JC, Falk RJ, Hu P, Xiao H. Pathogenesis of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Annu rev Pathol. 2013;8:139–60. doi:10.1146/annurev-pathol-011811-132453.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Jarrot PA, Kaplanski G. Pathogenesis of ANCA-associated vasculitis: an update. Autoimmunity reviews. 2016. doi:10.1016/j.autrev.2016.03.007.

  21. Savage CO. Pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. Clin Exp Immunol. 2011;164(Suppl 1):23–6. doi:10.1111/j.1365-2249.2011.04362.x.

    Article  PubMed  PubMed Central  Google Scholar 

  22. van Timmeren MM, Heeringa P. Pathogenesis of ANCA-associated vasculitis: recent insights from animal models. Curr Opin Rheumatol. 2012;24(1):8–14. doi:10.1097/BOR.0b013e32834bde57.

    Article  PubMed  Google Scholar 

  23. Kallenberg CG. Advances in pathogenesis and treatment of ANCA-associated vasculitis. Discov med. 2014;18(99):195–201.

    PubMed  Google Scholar 

  24. Jennette JC, Falk RJ. B cell-mediated pathogenesis of ANCA-mediated vasculitis. Semin Immunopathol. 2014;36(3):327–38. doi:10.1007/s00281-014-0431-y.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Abdulahad WH, Lamprecht P, Kallenberg CG. T-helper cells as new players in ANCA-associated vasculitides. Arthritis res Ther. 2011;13(4):236. doi:10.1186/ar3362.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Kindler V, Sappino AP, Grau GE, Piguet PF, Vassalli P. The inducing role of tumor necrosis factor in the development of bactericidal granulomas during BCG infection. Cell. 1989;56(5):731–40.

    Article  CAS  PubMed  Google Scholar 

  27. Noronha IL, Kruger C, Andrassy K, Ritz E, Waldherr R. In situ production of TNF-alpha, IL-1 beta and IL-2R in ANCA-positive glomerulonephritis. Kidney Int. 1993;43(3):682–92.

    Article  CAS  PubMed  Google Scholar 

  28. Nassonov EL, Samsonov MY, Tilz GP, Beketova TV, Semenkova EN, Baranov A, et al. Serum concentrations of neopterin, soluble interleukin 2 receptor, and soluble tumor necrosis factor receptor in Wegener's granulomatosis. J Rheumatol. 1997;24(4):666–70.

    CAS  PubMed  Google Scholar 

  29. • Xiao H, Schreiber A, Heeringa P, et al. Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic antibodies. Am J Pathol. 2007;170(1):52–64. This study was the first to demonstrate in a preclinical mouse model of anti-MPO ANCA vasculitis, a role for the alternative pathway of complement.

  30. Yuan J, Charles J, Zhao MH. Complement in antineutrophil cytoplasmic antibody-associated vasculitis. Clin Exp Nephrol. 2013;17:642–5.

    Article  CAS  PubMed  Google Scholar 

  31. Gou SJ, Yuan J, Chen M, Yu F, Zhao MH. Circulating complement activation in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis. Kidney Int. 2013;83(1):129–37. doi:10.1038/ki.2012.313.

    Article  CAS  PubMed  Google Scholar 

  32. Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum dis. 2007;66(5):605–17. doi:10.1136/ard.2006.062711.

    Article  CAS  PubMed  Google Scholar 

  33. de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, 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(10):670–80.

    Article  PubMed  Google Scholar 

  34. Walters GD, Willis NS, Craig JC. Interventions for renal vasculitis in adults. A Systematic Review BMC Nephrol. 2010;11:12. doi:10.1186/1471-2369-11-12.

    Article  PubMed  Google Scholar 

  35. Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. Journal of the American Society of Nephrology : JASN. 2007;18(7):2180–8. doi:10.1681/ASN.2007010090.

    Article  CAS  PubMed  Google Scholar 

  36. De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–9. doi:10.1002/art.21142.

    Article  CAS  PubMed  Google Scholar 

  37. Faurschou M, Westman K, Rasmussen N, de Groot K, Flossmann O, Hoglund P, et al. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012;64(10):3472–7. doi:10.1002/art.34547.

    Article  CAS  PubMed  Google Scholar 

  38. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J med. 2010;363(3):221–32. doi:10.1056/NEJMoa0909905.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J med. 2010;363(3):211–20. doi:10.1056/NEJMoa0909169.

    Article  CAS  PubMed  Google Scholar 

  40. Hu W, Liu C, Xie H, Chen H, Liu Z, Li L. Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2008;23(4):1307–12. doi:10.1093/ndt/gfm780.

    Article  CAS  Google Scholar 

  41. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Working Group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int. 2012;2:139–274.

    Article  Google Scholar 

  42. •• McGeoch L, Twilt M, Famorca L, Bakowsky V, Barra L, Benseler SM, et al. CanVasc recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitides. J Rheumatol. 2016;43(1):97–120. doi:10.3899/jrheum.150376. Evidence-based Canadian guidelines for the management of the ANCA vasculitides.

    Article  CAS  PubMed  Google Scholar 

  43. Furuta S, Jayne D. Emerging therapies in antineutrophil cytoplasm antibody-associated vasculitis. Curr Opin Rheumatol. 2014;26(1):1–6. doi:10.1097/BOR.0000000000000005.

    Article  CAS  PubMed  Google Scholar 

  44. Pepper RJ, Fabre MA, Pavesio C, Gaskin G, Jones RB, Jayne D, et al. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology. 2008;47(7):1104–5. doi:10.1093/rheumatology/ken175.

    Article  CAS  PubMed  Google Scholar 

  45. Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum dis. 2016;75(2):396–401. doi:10.1136/annrheumdis-2014-206095.

    Article  CAS  PubMed  Google Scholar 

  46. Moosig F, Gross WL, Herrmann K, Bremer JP, Hellmich B. Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern med. 2011;155(5):341–3. doi:10.7326/0003-4819-155-5-201109060-00026.

    Article  PubMed  Google Scholar 

  47. Lee T, Gasim A, Derebail VK, Chung Y, McGregor JG, Lionaki S, et al. Predictors of treatment outcomes in ANCA-associated vasculitis with severe kidney failure. Clinical Journal of the American Society of Nephrology : CJASN. 2014;9(5):905–13. doi:10.2215/CJN.08290813.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J med. 2008;359(26):2790–803. doi:10.1056/NEJMoa0802311.

    Article  CAS  PubMed  Google Scholar 

  49. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J med. 2003;349(1):36–44. doi:10.1056/NEJMoa020286.

    Article  CAS  PubMed  Google Scholar 

  50. Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. Jama. 2010;304(21):2381–8. doi:10.1001/jama.2010.1658.

    Article  CAS  PubMed  Google Scholar 

  51. Metzler C, Miehle N, Manger K, Iking-Konert C, de Groot K, Hellmich B, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology. 2007;46(7):1087–91. doi:10.1093/rheumatology/kem029.

    Article  CAS  PubMed  Google Scholar 

  52. • Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaitre O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J med. 2014;371(19):1771–80. doi:10.1056/NEJMoa1404231. This study showed the benefit of rituximab over azathioprine as maintenance therapy in ANCA vasculitis.

    Article  PubMed  Google Scholar 

  53. Wegener's Granulomatosis Etanercept Trial Research G. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J med. 2005;352(4):351–61. doi:10.1056/NEJMoa041884.

    Article  Google Scholar 

  54. Stegeman CA, Tervaert JW, De Jong MD, et al. Trimethoprim-sulfamethoxazole (co-Trimoxazole) for the prevention of relapses of Wegener's granulomatosis. N Engl J med. 1996;335(1):16–20.

    Article  CAS  PubMed  Google Scholar 

  55. Kemna M.J. DJ, Austen J., et al. ANCA as a predictor of relapse: useful in patients with renal involvement but not in patients with nonrenal disease. Journal of the American Society of Nephrology 2015;26(3):537–542.

  56. Jones RB, Ferraro AJ, Chaudhry AN, et al. A multicenter Survery of rituximab therapy for refractory Antineutrophil cytoplasmic antibody-associated Vasculitis. Arthritis Rheum. 2009;60(7):2156–68.

    Article  CAS  PubMed  Google Scholar 

  57. Cartin-Ceba R, Golbin JM, Keogh KA, et al. Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener’s). Arthritis Rheum. 2012;64(11):3770–8.

    Article  CAS  PubMed  Google Scholar 

  58. Basu B, Mahapatra TK, Mondal N. Favourable renal survival in paediatric microscopic polyangiitis: efficacy of a novel treatment algorithm. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2015;30(Suppl 1):i113–8. doi:10.1093/ndt/gfv016.

    Google Scholar 

  59. de Joode AA, Sanders JS, Stegeman CA. Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis. Clinical Journal of the American Society of Nephrology : CJASN. 2013;8(10):1709–17. doi:10.2215/CJN.01020113.

    Article  PubMed  PubMed Central  Google Scholar 

  60. • Noone DG, Twilt M, Hayes WN, Thorner PS, Benseler S, Laxer RM, et al. The new histopathologic classification of ANCA-associated GN and its association with renal outcomes in childhood. Clinical Journal of the American Society of Nephrology : CJASN. 2014;9(10):1684–91. doi:10.2215/CJN.01210214. This study validated the histopathologic classification for ANCA glomerulonephritis in pediatric patients and showed how it can predict renal outcomes.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Little MA, Hassan B, Jacques S, Game D, Salisbury E, Courtney AE, et al. Renal transplantation in systemic vasculitis: when is it safe? Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2009;24(10):3219–25. doi:10.1093/ndt/gfp347.

    Article  Google Scholar 

  62. Geetha D, Lee SM, Shah S, Rahman HM. Relevance of ANCA positivity at the time of renal transplantation in ANCA associated vasculitis. J Nephrol. 2015. doi:10.1007/s40620-015-0253-6.

  63. • Soliman M, Laxer R, Manson D, Yeung R, Doria AS. Imaging of systemic vasculitis in childhood. Pediatr Radiol. 2015;45(8):1110–25. doi:10.1007/s00247-015-3339-3. Pediatric specific guidelines to aid in the diagnosis of vasculitides for clinicians and radiologists.

    Article  PubMed  Google Scholar 

  64. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995;25(1):28–34.

    Article  CAS  PubMed  Google Scholar 

  65. Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145(4):517–22. doi:10.1016/j.jpeds.2004.06.046.

    Article  PubMed  Google Scholar 

  66. Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis study group database. Arthritis Rheum. 2010;62(2):616–26. doi:10.1002/art.27240.

    Article  PubMed  Google Scholar 

  67. Eleftheriou D, Batu ED, Ozen S, Brogan PA. Vasculitis in children. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 2015;30(Suppl 1):i94–103. doi:10.1093/ndt/gfu393.

    Google Scholar 

  68. Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years. Arthritis Rheum. 2013;65(9):2476–85. doi:10.1002/art.38024.

    Article  CAS  PubMed  Google Scholar 

  69. Mondal R, Sarkar S, Pal P, Nandi M, Hazra A, Sabui TK. Childhood polyarteritis nodosa: a prospective multicentre study from eastern India. Indian J Pediatr. 2014;81(4):371–4. doi:10.1007/s12098-013-1256-8.

    Article  PubMed  Google Scholar 

  70. Kumar L, Sarkar B, Singh S, Bajwa RP, Joshi K, Malik N. Polyarteritis nodosa—a few unusual findings. Indian Pediatr. 1996;33(6):459–64.

    CAS  PubMed  Google Scholar 

  71. Dillon MJ, Eleftheriou D, Brogan PA. Medium-size-vessel vasculitis. Pediatr Nephrol. 2010;25(9):1641–52. doi:10.1007/s00467-009-1336-1.

    Article  PubMed  Google Scholar 

  72. Yalcinkaya F, Ozcakar ZB, Kasapcopur O, Ozturk A, Akar N, Bakkaloglu A, et al. Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa. J Pediatr. 2007;151(6):675–8. doi:10.1016/j.jpeds.2007.04.062.

    Article  CAS  PubMed  Google Scholar 

  73. • Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, Padeh S, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J med. 2014;370(10):921–31. doi:10.1056/NEJMoa1307362. Recessive loss of function mutations in the gene encoding adenosine deaminase 2 (ADA2) can cause PAN.

    Article  PubMed  Google Scholar 

  74. Wright E, Dillon MJ, Tullus K. Childhood vasculitis and plasma exchange. Eur J Pediatr. 2007;166(2):145–51. doi:10.1007/s00431-006-0212-2.

    Article  PubMed  Google Scholar 

  75. Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French cooperative study Group for Vasculitides. Br J Rheumatol. 1997;36(12):1290–7.

    Article  CAS  PubMed  Google Scholar 

  76. de Kort SW, van Rossum MA, ten Cate R. Infliximab in a child with therapy-resistant systemic vasculitis. Clin Rheumatol. 2006;25(5):769–71. doi:10.1007/s10067-005-0071-7.

    Article  PubMed  Google Scholar 

  77. Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, et al. Biologic therapy in primary systemic vasculitis of the young. Rheumatology. 2009;48(8):978–86. doi:10.1093/rheumatology/kep148.

    Article  CAS  PubMed  Google Scholar 

  78. Besbas N, Ozen S, Saatci U, Topaloglu R, Tinaztepe K, Bakkaloglu A. Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children. Pediatr Nephrol. 2000;14(4):325–7.

    Article  CAS  PubMed  Google Scholar 

  79. Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu arteritis. Ann Intern med. 1994;120(11):919–29.

    Article  CAS  PubMed  Google Scholar 

  80. 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. doi:10.1186/1546-0096-12-21.

    Article  PubMed  PubMed Central  Google Scholar 

  81. Eleftheriou D, Varnier G, Dolezalova P, McMahon AM, Al-Obaidi M, Brogan PA. Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom. Arthritis res Ther. 2015;17:36. doi:10.1186/s13075-015-0545-1.

    Article  PubMed  PubMed Central  Google Scholar 

  82. Clemente G, Hilario MO, Lederman H, Silva CA, Sallum AM, Campos LM, et al. Takayasu arteritis in a Brazilian multicenter study: children with a longer diagnosis delay than adolescents. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S128–33.

    PubMed  Google Scholar 

  83. Brunner J, Feldman BM, Tyrrell PN, Kuemmerle-Deschner JB, Zimmerhackl LB, Gassner I, et al. Takayasu arteritis in children and adolescents. Rheumatology. 2010;49(10):1806–14. doi:10.1093/rheumatology/keq167.

    Article  PubMed  Google Scholar 

  84. Tann OR, Tulloh RM, Hamilton MC. Takayasu’s disease: a review. Cardiol Young. 2008;18(3):250–9. doi:10.1017/S1047951108002060.

    Article  PubMed  Google Scholar 

  85. Hijazi R, Chandar J, Nwobi O, Muneeruddin S, Zilleruelo G, Abitbol CL. Renal manifestations in toddlers with Takayasu’s arteritis and malignant hypertension. Pediatr Nephrol. 2009;24(6):1227–30. doi:10.1007/s00467-008-1088-3.

    Article  PubMed  Google Scholar 

  86. de Pablo P, Garcia-Torres R, Uribe N, Ramon G, Nava A, Silveira LH, et al. Kidney involvement in Takayasu arteritis. Clin Exp Rheumatol. 2007;25(1 Suppl 44):S10–4.

    PubMed  Google Scholar 

  87. Stern S, Clemente G, Reiff A, Ramos MP, Marzan KA, Terreri MT. Treatment of pediatric Takayasu arteritis with infliximab and cyclophosphamide: experience from an American-Brazilian cohort study. J Clin Rheumatol. 2014;20(4):183–8. doi:10.1097/RHU.0000000000000106.

    Article  PubMed  Google Scholar 

  88. Hoffman GS, Merkel PA, Brasington RD, Lenschow DJ, Liang P. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004;50(7):2296–304. doi:10.1002/art.20300.

    Article  CAS  PubMed  Google Scholar 

  89. van Timmeren MM, Heeringa P, Kallenberg CG. Infectious triggers for vasculitis. Curr Opin Rheumatol. 2014;26(4):416–23. doi:10.1097/BOR.0000000000000068.

    Article  PubMed  Google Scholar 

  90. Mason JC. Takayasu arteritis: surgical interventions. Curr Opin Rheumatol. 2015;27(1):45–52. doi:10.1097/BOR.0000000000000127.

    Article  PubMed  Google Scholar 

  91. Ozen S, Duzova A, Bakkaloglu A, Bilginer Y, Cil BE, Demircin M, et al. Takayasu arteritis in children: preliminary experience with cyclophosphamide induction and corticosteroids followed by methotrexate. J Pediatr. 2007;150(1):72–6. doi:10.1016/j.jpeds.2006.10.059.

    Article  CAS  PubMed  Google Scholar 

  92. Goel R, Kumar TS, Danda D, Joseph G, Jeyaseelan V, Surin AK, et al. Childhood-onset Takayasu arteritis—experience from a tertiary care center in South India. J Rheumatol. 2014;41(6):1183–9. doi:10.3899/jrheum.131117.

    Article  PubMed  Google Scholar 

  93. Filocamo G, Buoncompagni A, Viola S, Loy A, Malattia C, Ravelli A, et al. Treatment of Takayasu’s arteritis with tumor necrosis factor antagonists. J Pediatr. 2008;153(3):432–4. doi:10.1016/j.jpeds.2008.04.049.

    Article  CAS  PubMed  Google Scholar 

  94. Ladapo TA, Gajjar P, McCulloch M, Scott C, Numanoglu A, Nourse P. Impact of revascularization on hypertension in children with Takayasu’s arteritis-induced renal artery stenosis: a 21-year review. Pediatr Nephrol. 2015;30(8):1289–95. doi:10.1007/s00467-015-3049-y.

    Article  PubMed  Google Scholar 

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Correspondence to Damien Noone.

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Sally Kellett, Natasha Jawa, and Damien Noone each declare no potential conflicts of interest.

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Kellett, S., Jawa, N. & Noone, D. Renal Vasculitis in Childhood. Curr Pediatr Rep 5, 87–99 (2017). https://doi.org/10.1007/s40124-017-0138-3

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