Skip to main content
SpringerLink
Log in

Management of IgA Vasculitis with Nephritis

  • Current Opinion
  • Published:
Pediatric Drugs Aims and scope Submit manuscript

Abstract

Immunoglobulin A (IgA) vasculitis (IgAV), previously called Henoch–Schönlein purpura, is characterized by IgA-dominant immune deposits affecting small vessels and often involves the skin, gastrointestinal tract, joints, and kidneys. IgAV is the most common cause of systemic vasculitis in children. The long-term prognosis is dependent on renal involvement: IgAV with nephritis (IgAVN) can progress to renal failure. IgAVN is an inflammatory disease, providing a rationale for the use of corticosteroids. However, data supporting the use of corticosteroids in patients with established IgAVN of any severity remain limited, although most clinicians use them. Even in patients with severe forms of IgAVN, methylprednisolone pulses added to oral corticosteroids appears to improve renal outcomes. Considering the multihit hypothesis for the pathogenesis of IgAVN, involving many other immune agents, there is a strong rationale for the use of other immunosuppressive drugs in patients with IgAVN, including mycophenolic acid, cyclophosphamide, rituximab, calcineurin inhibitors, and complement inhibitors. Thus, these immunosuppressive treatments have also been evaluated in IgAVN, usually in corticosteroid-dependent or corticosteroid-resistant forms and in small retrospective studies. However, their efficacy has not been proven. Thus, the risk of progression to renal failure and the ongoing debate about the best management of IgAVN justifies the interest in investigating and identifying treatments that can potentially preserve renal function in patients with IgAVN. This review reports on the efficacy of the different drugs currently used for the treatment of IgAVN in adults and children.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010;69:798–806. https://doi.org/10.1136/ard.2009.116657.

    Article  PubMed  Google Scholar 

  2. Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch–Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet Lond Engl. 2002;360:1197–202. https://doi.org/10.1016/S0140-6736(02)11279-7.

    Article  Google Scholar 

  3. Chartapisak W, Opastirakul S, Hodson EM, et al. Interventions for preventing and treating kidney disease in Henoch–Schönlein Purpura (HSP). In: The Cochrane Collaboration (ed.) Cochrane Database of Systematic Reviews. Chichester: Wiley; 2009.

  4. Stewart M, Savage JM, Bell B, McCord B. Long term renal prognosis of Henoch–Schönlein purpura in an unselected childhood population. Eur J Pediatr. 1988;147:113–5.

    Article  CAS  Google Scholar 

  5. Goldstein AR, White RH, Akuse R, Chantler C. Long-term follow-up of childhood Henoch–Schönlein nephritis. Lancet Lond Engl. 1992;339:280–2.

    Article  CAS  Google Scholar 

  6. Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch–Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149:241–7. https://doi.org/10.1016/j.jpeds.2006.03.024.

    Article  CAS  PubMed  Google Scholar 

  7. Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein–Henoch purpura nephritis. Pediatr Nephrol. 1998;12(3):238–43. https://doi.org/10.1007/s004670050446.

    Article  CAS  PubMed  Google Scholar 

  8. Zaffanello M, Fanos V. Treatment-based literature of Henoch-Schönlein purpura nephritis in childhood. Pediatr Nephrol Berl Ger. 2009;24:1901–11. https://doi.org/10.1007/s00467-008-1066-9.

    Article  Google Scholar 

  9. Chapter 11: Henoch–Schönlein purpura nephritis. Kidney Int Suppl. 2012;2:218–20. https://doi.org/10.1038/kisup.2012.24.

  10. Lau KK, Suzuki H, Novak J, Wyatt RJ. Pathogenesis of Henoch-Schönlein purpura nephritis. Pediatr Nephrol Berl Ger. 2010;25:19–26. https://doi.org/10.1007/s00467-009-1230-x.

    Article  Google Scholar 

  11. Kiryluk K, Moldoveanu Z, Sanders JT, et al. Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis. Kidney Int. 2011;80:79–87. https://doi.org/10.1038/ki.2011.16.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Moura IC, Centelles MN, Arcos-Fajardo M, et al. Identification of the transferrin receptor as a novel immunoglobulin (Ig)A1 receptor and its enhanced expression on mesangial cells in IgA nephropathy. J Exp Med. 2001;194:417–25. https://doi.org/10.1084/jem.194.4.417.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Yanagihara T, Brown R, Hall S, et al. In vitro-generated immune complexes containing galactose-deficient IgA1 stimulate proliferation of mesangial cells. Results Immunol. 2012;2:166–72. https://doi.org/10.1016/j.rinim.2012.08.002.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Aleyd E, van Hout MWM, Ganzevles SH, et al. IgA enhances NETosis and release of neutrophil extracellular traps by polymorphonuclear cells via Fcα receptor I. J Immunol Baltim Md. 2014;192:2374–83. https://doi.org/10.4049/jimmunol.1300261.

    Article  CAS  Google Scholar 

  15. Yang Y-H, Wang SJ, Chuang Y-H, et al. The level of IgA antibodies to human umbilical vein endothelial cells can be enhanced by TNF-alpha treatment in children with Henoch–Schönlein purpura. Clin Exp Immunol. 2002;130:352–7. https://doi.org/10.1046/j.1365-2249.2002.01964.x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Belizna C, Duijvestijn A, Hamidou M, Tervaert JWC. Antiendothelial cell antibodies in vasculitis and connective tissue disease. Ann Rheum Dis. 2006;65:1545–50. https://doi.org/10.1136/ard.2005.035295.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Roos A, Rastaldi MP, Calvaresi N, et al. Glomerular activation of the lectin pathway of complement in IgA nephropathy is associated with more severe renal disease. J Am Soc Nephrol. 2006;17:1724–34. https://doi.org/10.1681/ASN.2005090923.

    Article  CAS  PubMed  Google Scholar 

  18. Kim SJ, Koo HM, Lim BJ, et al. Decreased circulating C3 levels and mesangial C3 deposition predict renal outcome in patients with IgA nephropathy. PLoS ONE. 2012;7:e40495. https://doi.org/10.1371/journal.pone.0040495.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Zhai Y-L, Meng S-J, Zhu L, et al. Rare variants in the complement factor H-related protein 5 gene contribute to genetic susceptibility to IgA nephropathy. J Am Soc Nephrol. 2016;27:2894–905. https://doi.org/10.1681/ASN.2015010012.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Heineke MH, Ballering AV, Jamin A, et al. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch–Schönlein purpura). Autoimmun Rev. 2017;16:1246–53. https://doi.org/10.1016/j.autrev.2017.10.009.

    Article  CAS  PubMed  Google Scholar 

  21. Counahan R, Winterborn MH, White RH, et al. Prognosis of Henoch–Schönlein nephritis in children. Br Med J. 1977;2:11–4. https://doi.org/10.1136/bmj.2.6078.11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Meadow SR, Glasgow EF, White RH, et al. Schönlein-Henoch nephritis. Q J Med. 1972;41:241–58.

    CAS  PubMed  Google Scholar 

  23. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch–Schönlein purpura: a retrospective cohort study. Lancet Lond Engl. 2002;360:666–70. https://doi.org/10.1016/S0140-6736(02)09835-5.

    Article  Google Scholar 

  24. Edström Halling S, Söderberg MP, Berg UB. Predictors of outcome in Henoch–Schönlein nephritis. Pediatr Nephrol Berl Ger. 2010;25:1101–8. https://doi.org/10.1007/s00467-010-1444-y.

    Article  Google Scholar 

  25. Wakaki H, Ishikura K, Hataya H, et al. Henoch–Schönlein purpura nephritis with nephrotic state in children: predictors of poor outcomes. Pediatr Nephrol Berl Ger. 2011;26:921–5. https://doi.org/10.1007/s00467-011-1827-8.

    Article  Google Scholar 

  26. Pillebout E, Thervet E, Hill G, et al. Henoch–Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol JASN. 2002;13:1271–8. https://doi.org/10.1097/01.asn.0000013883.99976.22.

    Article  PubMed  Google Scholar 

  27. Delbet J-D, Geslain G, Auger M, et al. Histological prognostic factors in children with Henoch–Schönlein purpura nephritis. Pediatr Nephrol Berl Ger. 2020;35:313–20. https://doi.org/10.1007/s00467-019-04363-y.

    Article  Google Scholar 

  28. Kim MJ, Schaub S, Molyneux K, et al. Effect of immunosuppressive drugs on the changes of serum galactose-deficient IgA1 in patients with IgA nephropathy. PLoS ONE. 2016;11:e0166830. https://doi.org/10.1371/journal.pone.0166830.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Delbet JD, Hogan J, Aoun B, et al. Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents. Pediatr Nephrol Berl Ger. 2017;32:1193–9. https://doi.org/10.1007/s00467-017-3604-9.

    Article  Google Scholar 

  30. Pozzi C, Bolasco PG, Fogazzi GB, et al. Corticosteroids in IgA nephropathy: a randomised controlled trial. Lancet Lond Engl. 1999;353:883–7. https://doi.org/10.1016/s0140-6736(98)03563-6.

    Article  CAS  Google Scholar 

  31. Manno C, Torres DD, Rossini M, et al. Randomized controlled clinical trial of corticosteroids plus ACE-inhibitors with long-term follow-up in proteinuric IgA nephropathy. Nephrol Dial Transpl. 2009;24:3694–701. https://doi.org/10.1093/ndt/gfp356.

    Article  CAS  Google Scholar 

  32. Lv J, Zhang H, Chen Y, et al. Combination therapy of prednisone and ACE inhibitor versus ACE-inhibitor therapy alone in patients with IgA nephropathy: a randomized controlled trial. Am J Kidney Dis. 2009;53:26–32. https://doi.org/10.1053/j.ajkd.2008.07.029.

    Article  CAS  PubMed  Google Scholar 

  33. Natale P, Palmer SC, Ruospo M, et al. Immunosuppressive agents for treating IgA nephropathy. Cochrane Database Syst Rev. 2020;3:CD003965. https://doi.org/10.1002/14651858.CD003965.pub3.

  34. Rauen T, Wied S, Fitzner C, et al. After ten years of follow-up, no difference between supportive care plus immunosuppression and supportive care alone in IgA nephropathy. Kidney Int. 2020;98:1044–52. https://doi.org/10.1016/j.kint.2020.04.046.

    Article  CAS  PubMed  Google Scholar 

  35. Dudley J, Smith G, Llewelyn-Edwards A, et al. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child. 2013;98:756–63. https://doi.org/10.1136/archdischild-2013-303642.

    Article  PubMed  Google Scholar 

  36. Kawasaki Y, Suzuki J, Nozawa R, et al. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch–Schönlein nephritis. Pediatrics. 2003;111:785–9. https://doi.org/10.1542/peds.111.4.785.

    Article  PubMed  Google Scholar 

  37. Katafuchi R, Ninomiya T, Mizumasa T, et al. The improvement of renal survival with steroid pulse therapy in IgA nephropathy. Nephrol Dial Transpl. 2008;23:3915–20. https://doi.org/10.1093/ndt/gfn394.

    Article  Google Scholar 

  38. Tudorache E, Azema C, Hogan J, et al. Even mild cases of paediatric Henoch-Schönlein purpura nephritis show significant long-term proteinuria. Acta Paediatr. 2015;104:843–8. https://doi.org/10.1111/apa.12723.

    Article  CAS  PubMed  Google Scholar 

  39. Ninchoji T, Kaito H, Nozu K, et al. Treatment strategies for Henoch-Schönlein purpura nephritis by histological and clinical severity. Pediatr Nephrol Berl Ger. 2011;26:563–9. https://doi.org/10.1007/s00467-010-1741-5.

    Article  Google Scholar 

  40. Praga M, Gutiérrez E, González E, et al. Treatment of IgA nephropathy with ACE inhibitors: a randomized and controlled trial. J Am Soc Nephrol JASN. 2003;14:1578–83.

    Article  CAS  Google Scholar 

  41. Coppo R, Peruzzi L, Amore A, et al. IgACE: a placebo-controlled, randomized trial of angiotensin-converting enzyme inhibitors in children and young people with IgA nephropathy and moderate proteinuria. J Am Soc Nephrol. 2007;18:1880–8. https://doi.org/10.1681/ASN.2006040347.

    Article  CAS  PubMed  Google Scholar 

  42. Li PK-T, Leung CB, Chow KM, et al. Hong Kong study using valsartan in IgA nephropathy (HKVIN): a double-blind, randomized, placebo-controlled study. Am J Kidney Dis. 2006;47:751–60. https://doi.org/10.1053/j.ajkd.2006.01.017.

    Article  CAS  PubMed  Google Scholar 

  43. Ronkainen J, Autio-Harmainen H, Nuutinen M. Cyclosporin A for the treatment of severe Henoch–Schönlein glomerulonephritis. Pediatr Nephrol Berl Ger. 2003;18:1138–42. https://doi.org/10.1007/s00467-003-1245-7.

    Article  Google Scholar 

  44. Shin JI, Park JM, Shin YH, et al. Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment. Scand J Rheumatol. 2005;34:392–5. https://doi.org/10.1080/03009740510026544.

    Article  CAS  PubMed  Google Scholar 

  45. Jauhola O, Ronkainen J, Autio-Harmainen H, et al. Cyclosporine A vs. methylprednisolone for Henoch-Schönlein nephritis: a randomized trial. Pediatr Nephrol Berl Ger. 2011;26:2159–66. https://doi.org/10.1007/s00467-011-1919-5.

    Article  Google Scholar 

  46. Kalliakmani P, Benou E, Goumenos DS. Cyclosporin A in adult patients with Henoch-Schönlein purpura nephritis and nephrotic syndrome; 5 case reports. Clin Nephrol. 2011;75:380–3. https://doi.org/10.5414/cn106553.

    Article  CAS  PubMed  Google Scholar 

  47. Ichiyama S, Matayoshi T, Kaneko T, et al. Successful multitarget therapy using prednisolone, mizoribine and tacrolimus for Henoch–Schönlein purpura nephritis in children. J Dermatol. 2017;44:e56–7. https://doi.org/10.1111/1346-8138.13614.

    Article  PubMed  Google Scholar 

  48. Zhang D-F, Hao G-X, Li C-Z, et al. Off-label use of tacrolimus in children with Henoch-Schönlein purpura nephritis: a pilot study. Arch Dis Child. 2018;103:772–5. https://doi.org/10.1136/archdischild-2017-313788.

    Article  PubMed  Google Scholar 

  49. Song Y-H, Cai G-Y, Xiao Y-F, et al. Efficacy and safety of calcineurin inhibitor treatment for IgA nephropathy: a meta-analysis. BMC Nephrol. 2017;18:61. https://doi.org/10.1186/s12882-017-0467-z.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Tarshish P, Bernstein J, Edelmann CM. Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol Berl Ger. 2004;19:51–6. https://doi.org/10.1007/s00467-003-1315-x.

    Article  Google Scholar 

  51. Pillebout E, Alberti C, Guillevin L, et al. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein Purpura. Kidney Int. 2010;78:495–502. https://doi.org/10.1038/ki.2010.150.

    Article  CAS  PubMed  Google Scholar 

  52. Audemard-Verger A, Terrier B, Dechartres A, et al. Characteristics and management of IgA Vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a French Multicenter Retrospective Survey. Arthritis Rheumatol Hoboken NJ. 2017;69:1862–70. https://doi.org/10.1002/art.40178.

    Article  CAS  Google Scholar 

  53. Bergstein J, Leiser J, Andreoli SP. Response of crescentic Henoch-Schoenlein purpura nephritis to corticosteroid and azathioprine therapy. Clin Nephrol. 1998;49:9–14.

    CAS  PubMed  Google Scholar 

  54. Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study. J Pediatr. 2000;136:370–5. https://doi.org/10.1067/mpd.2000.103448.

    Article  CAS  PubMed  Google Scholar 

  55. Devidayal SS, Kumar L, et al. Severe Henoch-Schönlein nephritis: resolution with azathioprine and steroids. Rheumatol Int. 2002;22:133–7. https://doi.org/10.1007/s00296-002-0208-9.

    Article  CAS  PubMed  Google Scholar 

  56. Sarcina C, Tinelli C, Ferrario F, et al. Changes in proteinuria and side effects of corticosteroids alone or in combination with azathioprine at different stages of IgA nephropathy. Clin J Am Soc Nephrol. 2016;11:973–81. https://doi.org/10.2215/CJN.02300215.

    Article  PubMed  PubMed Central  Google Scholar 

  57. Du Y, Hou L, Zhao C, et al. Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil. Pediatr Nephrol Berl Ger. 2012;27:765–71. https://doi.org/10.1007/s00467-011-2057-9.

    Article  Google Scholar 

  58. Nikibakhsh AA, Mahmoodzadeh H, Karamyyar M, et al. Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil. Saudi J Kidney Dis Transpl. 2014;25:858–63. https://doi.org/10.4103/1319-2442.135182.

    Article  PubMed  Google Scholar 

  59. Ren P, Han F, Chen L, et al. The combination of mycophenolate mofetil with corticosteroids induces remission of Henoch–Schönlein purpura nephritis. Am J Nephrol. 2012;36:271–7. https://doi.org/10.1159/000341914.

    Article  CAS  PubMed  Google Scholar 

  60. Han F, Chen L, Ren P, et al. Mycophenolate mofetil plus prednisone for inducing remission of Henoch-Schönlein purpura nephritis: a retrospective study. J Zhejiang Univ Sci B. 2015;16:772–9. https://doi.org/10.1631/jzus.B1400335.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Du B, Jia Y, Zhou W, et al. Efficacy and safety of mycophenolate mofetil in patients with IgA nephropathy: an update meta-analysis. BMC Nephrol. 2017;18:245. https://doi.org/10.1186/s12882-017-0647-x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Alberici F, Jayne DRW. Impact of rituximab trials on the treatment of ANCA-associated vasculitis. Nephrol Dial Transpl. 2014;29:1151–9. https://doi.org/10.1093/ndt/gft318.

    Article  CAS  Google Scholar 

  63. Pillebout E, Rocha F, Fardet L, et al. Successful outcome using rituximab as the only immunomodulation in Henoch–Schonlein purpura: case report. Nephrol Dial Transpl. 2011;26:2044–6. https://doi.org/10.1093/ndt/gfr137.

    Article  CAS  Google Scholar 

  64. Donnithorne KJ, Atkinson TP, Hinze CH, et al. Rituximab therapy for severe refractory chronic Henoch–Schönlein purpura. J Pediatr. 2009;155:136–9. https://doi.org/10.1016/j.jpeds.2008.12.049.

    Article  CAS  PubMed  Google Scholar 

  65. 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;65:186–92. https://doi.org/10.1007/s12026-016-8827-5.

    Article  CAS  PubMed  Google Scholar 

  66. Maritati F, Fenoglio R, Pillebout E, et al. Brief Report: Rituximab for the Treatment of Adult-Onset IgA Vasculitis (Henoch-Schönlein). Arthritis Rheumatol Hoboken NJ. 2018;70:109–14. https://doi.org/10.1002/art.40339.

    Article  CAS  Google Scholar 

  67. Lafayette RA, Canetta PA, Rovin BH, et al. A randomized, controlled trial of Rituximab in IgA nephropathy with proteinuria and renal dysfunction. J Am Soc Nephrol. 2017;28:1306–13. https://doi.org/10.1681/ASN.2016060640.

    Article  CAS  PubMed  Google Scholar 

  68. Gianviti A, Trompeter RS, Barratt TM, et al. Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis. Arch Dis Child. 1996;75:186–90.

    Article  CAS  Google Scholar 

  69. Hattori M, Ito K, Konomoto T, et al. Plasmapheresis as the sole therapy for rapidly progressive Henoch–Schönlein purpura nephritis in children. Am J Kidney Dis. 1999;33:427–33.

    Article  CAS  Google Scholar 

  70. Schärer K, Krmar R, Querfeld U, et al. Clinical outcome of Schönlein–Henoch purpura nephritis in children. Pediatr Nephrol Berl Ger. 1999;13:816–23. https://doi.org/10.1007/s004670050707.

    Article  Google Scholar 

  71. Shenoy M, Ognjanovic MV, Coulthard MG. Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol Berl Ger. 2007;22:1167–71. https://doi.org/10.1007/s00467-007-0498-y.

    Article  Google Scholar 

  72. Augusto J-F, Sayegh J, Delapierre L, et al. Addition of plasma exchange to glucocorticosteroids for the treatment of severe Henoch-Schönlein purpura in adults: a case series. Am J Kidney Dis. 2012;59:663–9. https://doi.org/10.1053/j.ajkd.2011.12.015.

    Article  CAS  PubMed  Google Scholar 

  73. Lee KH, Hong SH, Jun J, et al. Treatment of refractory IgA vasculitis with dapsone: a systematic review. Clin Exp Pediatr. 2020;63:158–63. https://doi.org/10.3345/kjp.2019.00514.

    Article  CAS  PubMed  Google Scholar 

  74. Iqbal H, Evans A. Dapsone therapy for Henoch–Schönlein purpura: a case series. Arch Dis Child. 2005;90:985–6. https://doi.org/10.1136/adc.2004.061598.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  75. Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. Report of three cases. J Am Acad Dermatol. 1987;16:772–8. https://doi.org/10.1016/s0190-9622(87)70100-5.

    Article  CAS  PubMed  Google Scholar 

  76. Rizk DV, Maillard N, Julian BA, et al. The emerging role of complement proteins as a target for therapy of IgA nephropathy. Front Immunol. 2019;10:504. https://doi.org/10.3389/fimmu.2019.00504.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Lafayette RA, Rovin BH, Reich HN, et al. Safety, tolerability and efficacy of Narsoplimab, a Novel MASP-2 Inhibitor for the treatment of IgA Nephropathy. Kidney Int Rep. 2020;5:2032–41. https://doi.org/10.1016/j.ekir.2020.08.003.

    Article  PubMed  PubMed Central  Google Scholar 

  78. Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis. 2006;47:993–1003. https://doi.org/10.1053/j.ajkd.2006.02.178.

    Article  PubMed  Google Scholar 

  79. Hennies I, Gimpel C, Gellermann J, et al. Presentation of pediatric Henoch-Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing. Pediatr Nephrol Berl Ger. 2018;33:277–86. https://doi.org/10.1007/s00467-017-3794-1.

    Article  Google Scholar 

  80. de Graeff N, Groot N, Brogan P, et al. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides—the SHARE initiative. Rheumatol Oxf Engl. 2020;59:919. https://doi.org/10.1093/rheumatology/keaa057.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Pediatric Nephrology Unit, Trousseau Hospital, APHP.6, DMU Origyne, Paris, France

    Jean-Daniel Delbet, Cyrielle Parmentier, Claire Herbez Rea, Antoine Mouche & Tim Ulinski

  2. Sorbonne Université, Paris, France

    Jean-Daniel Delbet, Cyrielle Parmentier, Claire Herbez Rea, Antoine Mouche & Tim Ulinski

  3. Department of Pediatric Nephrology, Armand Trousseau Hospital, 26 Avenue du Docteur Arnold Netter, 75012, Paris, France

    Jean-Daniel Delbet

Authors
  1. Jean-Daniel Delbet
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cyrielle Parmentier
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Claire Herbez Rea
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Antoine Mouche
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Tim Ulinski
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jean-Daniel Delbet.

Ethics declarations

Funding

No sources of funding were used to conduct this study or prepare this manuscript.

Conflict of Interest

Jean-Daniel Delbet, Cyrielle Parmentier, Claire Herbez Rea, Antoine Mouche, and Tim Ulinski have no conflicts of interest that are directly relevant to the content of this article.

Availability of Data and Material

Not applicable.

Ethics Approval

Not applicable.

Informed Consent

Not applicable.

Author Contributions

J-DD wrote the first version of the manuscript, examined the appropriate literature, coordinated the different steps of the submission process, and responded to the reviewers’ remarks. CP, AM, and CH participated in the literature search and discussion of the manuscript. TU revised and discussed the manuscript and validated the final version and the responses to the reviewers’ remarks.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Delbet, JD., Parmentier, C., Herbez Rea, C. et al. Management of IgA Vasculitis with Nephritis. Pediatr Drugs 23, 425–435 (2021). https://doi.org/10.1007/s40272-021-00464-0

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40272-021-00464-0

Search

Navigation