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Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management

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Abstract

Background

Henoch-Schönlein purpura (HSP) is one of the most common vasculitides in children. It is manifested by skin purpura, arthritis, abdominal pain, renal involvement, etc. Typically, HSP is considered to be self-limiting, although renal involvement (HSP purpura nephritis, HSPN) is the principal cause of morbidity from this disease. For this reason, it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN. In this article, we review the updated pathophysiology and treatment strategies for HSPN.

Data sources

We searched databases including PubMed, Elsevier and Wanfang for the following key words: Henoch-Schönlein purpura, nephritis, mechanism and treatment, and we selected those publications written in English that we judged to be relevant to the topic of this review.

Results

Based on the data present in the literature, we reviewed the following topics: 1) the possible pathogenesis of HSPN: several studies suggest that immunoglobulin A immune complexes deposit in the mesangium and induce renal injury; 2) multiple-drug treatment for HSPN: although there have been few evidence-based treatment strategies for HSPN, several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity.

Conclusions

HSPN is a severe disease of childhood. To better understand this disease, detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.

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References

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

    Article  PubMed  Google Scholar 

  2. Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 2001;80:279–290.

    Article  Google Scholar 

  3. Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 1999;78:395–409.

    Article  CAS  Google Scholar 

  4. Nielsen HE. Epidemiology of Schönlein-Henoch purpura. Acta Paediatr Scand 1988;77:125–131.

    Article  CAS  PubMed  Google Scholar 

  5. 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;147:113–115.

    Article  CAS  PubMed  Google Scholar 

  6. Yoshikawa N, Ito H, Yoshiya K, Nakahara C, Yoshiara S, Hasegawa O, et al. Henoch-Schoenlein nephritis and IgA nephropathy in children: a comparison of clinical course. Clin Nephrol 1987;27:233–237.

    CAS  PubMed  Google Scholar 

  7. Counahan R, Winterborn MH, White RH, Heaton JM, Meadow SR, Bluett NH, et al. Prognosis of Henoch-Schönlein nephritis in children. Br Med J 1977;2:11–14.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Bunchman TE, Mauer SM, Sibley RK, Vernier RL. Anaphylactoid purpura: characteristics of 16 patients who progressed to renal failure. Pediatr Nephrol 1988;2:393–397.

    Article  CAS  PubMed  Google Scholar 

  9. Saulsbury FT. Epidemiology of Henoch-Schönlein purpura. Cleve Clin J Med 2002;69Suppl 2:SII87–SII89.

    PubMed  Google Scholar 

  10. Davin JC, Ten Berge IJ, Weening JJ. What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis?. Kidney Int 2001;59:823–834.

    Article  CAS  PubMed  Google Scholar 

  11. Knight JF. The rheumatic poison: a survey of some published investigations of the immunopathogenesis of Henoch-Schönlein purpura. Pediatr Nephrol 1990;4:533–541.

    Article  CAS  PubMed  Google Scholar 

  12. Saulsbury FT. IgA rheumatoid factor in Henoch-Schönlein purpura. J Pediatr 1986;108:71–76.

    Article  CAS  PubMed  Google Scholar 

  13. Fervenza FC. Henoch-Schonlein purpura nephritis. Int J Dermatol 2003;42:170–177.

    Article  PubMed  Google Scholar 

  14. Pohl M. Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2014 Apr 15. [Epub ahead of print]

    Google Scholar 

  15. Levinsky RJ, Barratt TM. IgA immune complexes in Henoch-Schönlein purpura. Lancet 1979;2:1100–1103.

    Article  CAS  PubMed  Google Scholar 

  16. Renfrow MB, Mackay CL, Chalmers MJ, Julian BA, Mestecky J, Kilian M, et al. Analysis of O-glycan heterogeneity in IgA1 myeloma proteins by Fourier transform ion cyclotron resonance mass spectrometry: implications for IgA nephropathy. Anal Bioanal Chem 2007;389:1397–1407.

    Article  CAS  PubMed  Google Scholar 

  17. Tarelli E, Smith AC, Hendry BM, Challacombe SJ, Pouria S. Human serum IgA1 is substituted with up to six O-glycans as shown by matrix assisted laser desorption ionisation time-of-flight mass spectrometry. Carbohydr Res 2004;339:2329–2335.

    Article  CAS  PubMed  Google Scholar 

  18. Field MC, Dwek RA, Edge CJ, Rademacher TW. O-linked oligosaccharides from human serum immunoglobulin A1. Biochem Soc Trans 1989;17:1034–1035.

    CAS  PubMed  Google Scholar 

  19. Baenziger J, Kornfeld S. Structure of the carbohydrate units of IgA1 immunoglobulin. II. Structure of the O-glycosidically linked oligosaccharide units. J Biol Chem 1974;249:7270–7281.

    CAS  PubMed  Google Scholar 

  20. Novak J, Moldoveanu Z, Renfrow MB, Yanagihara T, Suzuki H, Raska M, et al. IgA nephropathy and Henoch-Schoenlein purpura nephritis: aberrant glycosylation of IgA1, formation of IgA1-containing immune complexes, and activation of mesangial cells. Contrib Nephrol 2007;157:134–138.

    Article  CAS  PubMed  Google Scholar 

  21. Lau KK, Wyatt RJ, Moldoveanu Z, Tomana M, Julian BA, Hogg RJ, et al. Serum levels of galactose-deficient IgA in children with IgA nephropathy and enoch-Schönlein purpura. Pediatr Nephrol 2007;22:2067–2072.

    Article  PubMed  Google Scholar 

  22. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosylation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant 1998;13:930–934.

    Article  CAS  PubMed  Google Scholar 

  23. Gharavi AG, Moldoveanu Z, Wyatt RJ, Barker CV, Woodford SY, Lifton RP, et al. Aberrant IgA1 glycosylation is inherited in familial and sporadic IgA nephropathy. J Am Soc Nephrol 2008;19:1008–1014.

    Article  PubMed Central  PubMed  Google Scholar 

  24. Tomana M, Matousovic K, Julian BA, Radl J, Konecny K, Mestecky J. Galactose-deficient IgA1 in sera of IgA nephropathy patients is present in complexes with IgG. Kidney Int 1997;52:509–516.

    Article  CAS  PubMed  Google Scholar 

  25. Novak J, Vu HL, Novak L, Julian BA, Mestecky J, Tomana M, et al. Interactions of human mesangial cells with IgA and IgAcontaining immune complexes. Kidney Int 2002;62:465–475.

    Article  CAS  PubMed  Google Scholar 

  26. Oortwijn BD, Eijgenraam JW, Rastaldi MP, Roos A, Daha MR, van Kooten C. The role of secretory IgA and complement in IgA nephropathy. Semin Nephrol 2008;28:58–65.

    Article  CAS  PubMed  Google Scholar 

  27. Wyatt RJ, Kanayama Y, Julian BA, Negoro N, Sugimoto S, Hudson EC, et al. Complement activation in IgA nephropathy. Kidney Int 1987;31:1019–1023.

    Article  CAS  PubMed  Google Scholar 

  28. Moura IC, Benhamou M, Launay P, Vrtovsnik F, Blank U, Monteiro RC, et al. The glomerular response to IgA deposition in IgA nephropathy. Semin Nephrol 2008;28:88–95.

    Article  CAS  PubMed  Google Scholar 

  29. Masuda M, Nakanishi K, Yoshizawa N, Iijima K, Yoshikawa N. Group A streptococcal antigen in the glomeruli of children with Henoch-Schönlein nephritis. Am J Kidney Dis 2003;41:366–370.

    Article  PubMed  Google Scholar 

  30. Chen Y, Zhou JH, Wu HS, Wang HW. Role of mast cells and eosinophil cationic protein in the pathogenesis of Henoch-Schönlein purpura nephritis. Zhonghua Er Ke Za Zhi 2006;44:407–410. [In Chinese]

    PubMed  Google Scholar 

  31. Kawasaki Y, Hosoya M, Suzuki H. Possible pathologenic role of interleukin-5 and eosino cationic protein in Henoch-Schönlein purpura nephritis. Pediatr Int 2005;47:512–517.

    Article  CAS  PubMed  Google Scholar 

  32. Namgoong MK, Lim BK, Kim JS. Eosinophil cationic protein in Henoch-Schönlein purpura and in IgA nephropathy. Pediatr Nephrol 1997;11:703–706.

    Article  CAS  PubMed  Google Scholar 

  33. Kawasaki Y, Imaizumi T, Matsuura H, Ohara S, Takano K, Suyama K, et al. Renal expression of alpha-smooth muscle actin and c-Met in children with Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2008;23:913–919.

    Article  PubMed  Google Scholar 

  34. Chapter 11: Henoch-Schönlein purpura nephritis. Kidney Int Suppl (2011) 2012;2:218–220.

  35. Davin JC, Coppo R. Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2013;28:1897–1903.

    Article  PubMed  Google Scholar 

  36. Park JM, Won SC, Shin JI, Yim H, Pai KS. Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria. Pediatr Nephrol 2011;26:411–417.

    Article  PubMed  Google Scholar 

  37. Du Y, Hou L, Zhao C, Han M, Wu Y. Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil. Pediatr Nephrol 2012;27:765–771.

    Article  PubMed  Google Scholar 

  38. Fuentes Y, Valverde S, Valesquez-Jones L. Comparison of azathioprine vs mofetil mycophenolate for Henoch-Schönlein nephritis treatment (abstract). Pediatr Nephrol 2010;25:1802.

    Google Scholar 

  39. Wu L, Mao J, Jin X, Fu H, Shen H, Wang J, et al. Efficacy of triptolide for children with moderately severe Henoch-Schönlein purpura nephritis presenting with nephrotic range proteinuria: a prospective and controlled study in China. Biomed Res Int 2013;2013:292865.

    PubMed Central  PubMed  Google Scholar 

  40. Pillebout E, Rocha F, Fardet L, Rybojad M, Verine J, Glotz D. Successful outcome using rituximab as the only immunomodulation in Henoch-Schönlein purpura: case report. Nephrol Dial Transplant 2011;26:2044–2046.

    Article  CAS  PubMed  Google Scholar 

  41. Donnithorne KJ, Atkinson TP, Hinze CH, Nogueira JB, Saeed SA, Askenazi DJ, et al. Rituximab therapy for severe refractory chronic Henoch-Schonlein purpura. J Pediatr 2009;155:136–139.

    Article  CAS  PubMed  Google Scholar 

  42. Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schönlein Purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol 2001;21:128–133.

    Article  CAS  PubMed  Google Scholar 

  43. Kawasaki Y, Suyama K, Hashimoto K, Hosoya M. Methylprednisolone pulse plus mizoribine in children with Henoch-Schoenlein purpura nephritis. Clin Rheumatol 2011;30:529–535.

    Article  PubMed  Google Scholar 

  44. Kawasaki Y, Suzuki J, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schoenlein nephritis: a clinical and histopathological study. Nephrol Dial Transplant 2004;19:858–864.

    Article  CAS  PubMed  Google Scholar 

  45. Kawasaki Y, Suzuki J, Murai M, Takahashi A, Isome M, Nozawa R, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schönlein nephritis. Pediatr Nephrol 2004;19:920–923.

    Article  PubMed  Google Scholar 

  46. Davin JC. Henoch-Schönlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol 2011;6:679–689.

    Article  PubMed  Google Scholar 

  47. Donghi D, Schanz U, Sahrbacher U, Recher M, Trüeb RM, Müllhaupt B, et al. Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage. Dermatology 2009;219:167–170.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  49. Zaffanello M, Brugnara M, Franchini M, Fanos V. Adjuvant treatments for Henoch-Schönlein purpura nephritis in children: A systematic review. Curr Ther Res Clin Exp 2009;70:254–265.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  50. Kawasaki Y, Suyama K, Matsumoto A, Takano K, Hashimoto K, Suzuki S, et al. Efficacy of tonsillectomy plus methylprednisolone pulse therapy for a child with Henoch-Schoenlein purpura nephritis. Tohoku J Exp Med 2007;211:291–295.

    Article  PubMed  Google Scholar 

  51. Kanai H, Sawanobori E, Kobayashi A, Matsushita K, Sugita K, Higashida K. Early treatment with methylprednisolone pulse therapy combined with tonsillectomy for heavy proteinuric henoch-schönlein purpura nephritis in children. Nephron Extra 2011;1:101–111.

    Article  PubMed Central  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Nephrology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou, 310003, China

    Jun-Yi Chen & Jian-Hua Mao

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Correspondence to Jian-Hua Mao.

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Chen, JY., Mao, JH. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr 11, 29–34 (2015). https://doi.org/10.1007/s12519-014-0534-5

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