Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Treatment of Severe Henoch-Schoenlein Purpura Nephritis in Children

소아의 심한 헤노흐-쇤라인 자반증의 치료

  • Shin, Jae-Il (The Institute of Kidney Disease, Department of Pediatrics Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Lee, Jae-Seung (The Institute of Kidney Disease, Department of Pediatrics Yonsei University College of Medicine, Severance Children's Hospital)
  • 신재일 (연세대학교 의과대학 소아과학교실 신장질환 연구소, 세브란스 어린이병원) ;
  • 이재승 (연세대학교 의과대학 소아과학교실 신장질환 연구소, 세브란스 어린이병원)
  • Received : 2010.03.28
  • Accepted : 2010.04.12
  • Published : 2010.04.30
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Abstract

The overall prognosis of Henoch-Schoenlein purpura (HSP) is favorable, but severe nephritis has a high risk of progression to end stage renal failure. Recent studies emphasize the importance of early treatment in children with severe HSP nephritis, but the treatment of severe HSP nephritis still remains controversial due to the rarity of randomized controlled studies in this field. Nevertheless, several intensive therapies, such as intravenous high-dose methylprednisolone pulse, immunosuppressive/cytotoxic drugs, fibrinolytic therapy, anticoagulants, antiplatelet agent and plasma exchange, have been used in children with severe HSP nephritis. In this review, we focus on the treatment of severe HSP nephritis in children.

헤노흐-쇤라인 자반증의 전반적인 예후는 양호하나 심한 신염의 경우 말기신부전으로 진행될 위험이 높다. 최근 연구는 심한 헤노흐-쇤라인 자반증 신염을 가진 소아에서 조기 치료의 중요성을 강조하고 있으나 심한 자반증 신염의 치료는 맹검 대조 연구가 드물어 여전히 논쟁의 여지가 있다. 그럼에도, 정맥 고용량 메틸프레드니솔론 충격요법, 면역억제/세포독성 약제, 섬유소용해 치료, 항응고제, 항혈소판제, 혈장교환술같은 여러 강력한 치료가 심한 자반증 신염을 가진 소아에서 사용되어 왔다. 이 종설에서는 심한 자반증 신염을 가진 소아의 치료를 중점적으로 기술하였다.

Keywords

References

  1. Davin J-C, Weening JJ. Henoch-Schonlein purpura nephritis: an update. Eur J Pediatr 2001;160:689-95. https://doi.org/10.1007/s004310100841
  2. Koskimies O, Mir S, Rapola J, Vilska J. Henoch-Schönlein nephritis: long-term prognosis of unselected patients. Arch Dis Child 1981;56:482-4. https://doi.org/10.1136/adc.56.6.482
  3. Goldstein AR, White RHR, Akuse R, Chantler C. Long-term follow-up of childhood Henoch- Schönlein nephritis. Lancet 1992;339: 280-2. https://doi.org/10.1016/0140-6736(92)91341-5
  4. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schonlein purpura. A retrospective cohort study. Lancet 2002;360:666-70. https://doi.org/10.1016/S0140-6736(02)09835-5
  5. Chang WL, Yang YH, Wang LC, Lin YT, Chiang BL. Renal manifestations in Henoch- Schonlein purpura: a 10-year clinical study. Pediatr Nephrol 2005;20:1269-72. https://doi.org/10.1007/s00467-005-1903-z
  6. Rostoker G. Schonlein-henoch purpura in children and adults: diagnosis, pathophysiology and management. BioDrugs 2001;15: 99-138. https://doi.org/10.2165/00063030-200115020-00004
  7. Kaku Y, Nohara K, Honda S. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of prognostic factors. Kidney Int 1998;53:1755-9. https://doi.org/10.1046/j.1523-1755.1998.00915.x
  8. Sano H, Izumida M, Shimizu H, Ogawa Y. Risk factors of renal involvement and significant proteinuria in Henoch-Schonlein purpura. Eur J Pediatr 2002;161:196-201. https://doi.org/10.1007/s00431-002-0922-z
  9. Shin JI, Park JM, Shin YH, Hwang DH, Kim JH, Lee JS. Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Schonlein purpura. Scand J Rheumatol 2006;35:56-60. https://doi.org/10.1080/03009740510026841
  10. Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 2005;90: 916-20. https://doi.org/10.1136/adc.2005.074641
  11. Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch nephritis. Pediatr Nephrol 1998;12:238-43. https://doi.org/10.1007/s004670050446
  12. Wyatt RJ, Hogg RJ. Evidence-based assessment of treatment options for children with IgA nephropathies. Pediatr Nephrol 2001;16:156-67. https://doi.org/10.1007/s004670000517
  13. Yoshikawa N, Tanaka R, Iijima K. Pathophysiology and treatment of IgA nephropathy in children. Pediatr Nephrol 2001;16:446-57. https://doi.org/10.1007/s004670100582
  14. Tarshish P, Bernstein J, Edelmann CM Jr. Henoch-Schonlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 2004;19:51-6. https://doi.org/10.1007/s00467-003-1315-x
  15. Rieu P, Noel LH. Henoch-Schonlein nephritis in children and adults. Morphological features and clinicopathological correlations. Ann Med Interne (Paris) 1999;150:151-9.
  16. Schonlein nephritis: clinical findings related to renal function and morphology. Pediatr Nephrol 2005;20:46-51. https://doi.org/10.1007/s00467-004-1650-6
  17. Ronkainen J, Ala-Houhala M, Huttunen NP, Jahnukainen T, Koskimies O, ormala T, et al. Outcome of Henoch-Schonlein nephritis with nephrotic-range proteinuria. Clin Nephrol 2003;60:80-4. https://doi.org/10.5414/CNP60080
  18. Ruggenenti P, Schieppati A, Remuzzi G. Progression, remission, regression of chronic renal diseases. Lancet 2001;357:1601-8. https://doi.org/10.1016/S0140-6736(00)04728-0
  19. Shin JI, Park JM, Kim JH, Lee JS, Jeong HJ. Factors affecting histological regression of crescentic Henoch-Schonlein nephritis in children. Pediatr Nephrol 2006;21:54-9. https://doi.org/10.1007/s00467-005-2068-5
  20. Meadow SR, Glasgow EF, White RHR, Moncrief W, Cameron JS, Ogg CS. Schonlein-Henoch nephritis. Q J Med 1972;41:241-58.
  21. Kawasaki Y, Suzuki J, Sakai N, Nemoto K, Nozawa R, Suzuki S, et al. Clinical and pathological features of children with Henoch Schoenlein purpura nephritis: risk factors associated with poor prognosis. Clin Nephrol 2003;60:153-60. https://doi.org/10.5414/CNP60153
  22. Mir S, Yavascan O, Mutlubas F, Yeniay B, Sonmez F. Clinical outcome in children with Henoch-Schonlein nephritis. Pediatr Nephrol 2007;22:64-70. https://doi.org/10.1007/s00467-006-0278-0
  23. Algoet C, Proesmans W. Renal biopsy 2-9 years after Henoch-Schonlein purpura. Pediatr Nephrol 2003;18:471-3.
  24. Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schonlein Purpura glomerulone phritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol 2001;21:128-33. https://doi.org/10.1159/000046235
  25. Tanaka H, Suzuki K, Nakahata T, Ito E, Waga S. Early treatment with oral immunosuppressants in severe proteinuric purpura nephritis. Pediatr Nephrol 2003;18:347-50.
  26. Oner A, Tinaztepe K, Erdogan O. The effect of triple therapy on rapidly progressive type of Henoch-Schonlein nephritis. Pediatr Nephrol 1995;9:6-10. https://doi.org/10.1007/BF00858954
  27. Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N. Multiple combined therapy for severe Henoch-Schonlein nephritis in children. Pediatr Nephrol 1998;12:244-8. https://doi.org/10.1007/s004670050447
  28. Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch-Schonlein nephritis. Pediatrics 2003;111:785-9. https://doi.org/10.1542/peds.111.4.785
  29. 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: 858-64. https://doi.org/10.1093/ndt/gfg617
  30. Bergstein J, Leiser J, Andreoli SP. Response of crescentic Henoch-Schoenlein purpura nephritis to corticosteroid and azathioprine therapy. Clin Nephrol 1998;49:9-14.
  31. Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch- Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathological study. J Pediatr 2000;136: 370-5. https://doi.org/10.1067/mpd.2000.103448
  32. Singh S, Devidayal, Kumar L, Joshi K, Minz RW, Datta U. Severe Henoch-Schonlein nephritis: resolution with azathioprine and steroids. Rheumatol Int 2002;22:133-7. https://doi.org/10.1007/s00296-002-0208-9
  33. Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Kim PK, et al. Can azathioprine and steroids alter the progression of severe Henoch-Schonlein nephritis in children? Pediatr Nephrol 2005;20:1087-92. https://doi.org/10.1007/s00467-005-1869-x
  34. Ronkainen J, Autio-Harmainen H, Nuutinen M. Cyclosporin A for the treatment of severe Henoch-Schonlein glomerulonephritis. Pediatr Nephrol 2003;18:1138-42. https://doi.org/10.1007/s00467-003-1245-7
  35. Shin JI, Park JM, Shin YH, Kim JH, Kim PK, Lee JS, et al. Cyclosporin A therapy for severe Henoch-Schonlein nephritis with nephrotic syndrome. Pediatr Nephrol 2005; 20:1093-7. https://doi.org/10.1007/s00467-005-1864-2
  36. Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Jeong HJ. Henoch-Schonlein 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
  37. Shin JI, Park JM, Lee JS, Kim JH, Kim PK, Jeong HJ. Successful use of cyclosporin A in severe Schonlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine. Clin Rheumatol 2006;25: 759-60. https://doi.org/10.1007/s10067-005-0066-4
  38. Shin JI, Park JM, Shin YH, Lee JS, Jeong HJ, Kim HS. Serum IgA/C3 ratio may be a useful marker of disease activity in severe Henoch-Schonlein nephritis. Nephron Clin Pract 2005;101:c72-8. https://doi.org/10.1159/000086225
  39. Watanabe T, Takahashi S, Nakajo S, Hamasaki M. Pathological improvement of IgA nephropathy and Henoch-Schonlein purpura nephritis with urokinase therapy. Acta Pae diatr Jpn 1996;38:622-8. https://doi.org/10.1111/j.1442-200X.1996.tb03720.x
  40. Shin JI, Park JM, Shin YH, Lee JS, Jeong HJ. Role of mesangial fibrinogen deposition in the pathogenesis of crescentic Henoch-Schonlein nephritis in children. J Clin Pathol 2005;58:1147-51. https://doi.org/10.1136/jcp.2005.027409
  41. Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M. Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schonlein purpura nephritis in children. Am J Kidney Dis 1999;33:427-33. https://doi.org/10.1016/S0272-6386(99)70178-2
  42. Scharer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F. Clinical outcome of Schonlein-Henoch purpura nephritis in children. Pediatr Nephrol 1999;13:816-23. https://doi.org/10.1007/s004670050707
  43. Kawasaki Y, Suzuki J, Murai M, Takahashi A, Isome M, Nozawa R, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schonlein nephritis. Pediatr Nephrol 2004; 19:920-3.
  44. Wright E, Dillon MJ, Tullus K. Childhood vasculitis and plasma exchange. Eur J Pediatr 2007;166:145-51.
  45. Wortmann SB, Fiselier TJ, Van De Kar NC, Aarts RA, Warris A, Draaisma JM. Refractory severe intestinal vasculitis due to Henoch-Schonlein purpura: successful treatment with plasmapheresis. Acta Paediatr 2006;95:622-3. https://doi.org/10.1080/08035250500509180
  46. Wen YK, Yang Y, Chang CC. Cerebral vasculitis and intracerebral hemorrhage in Henoch-Schonlein purpura treated with plasmapheresis. Pediatr Nephrol 2005;20: 223-5. https://doi.org/10.1007/s00467-004-1673-z
  47. Yamamoto-Shuda Y, Nakayama K, Saito T, Natori Y. Therapeutic effect of glucocorticoid on experimental crescentic glomerulonephritis. J Lab Clin Med 1999;134:410-8. https://doi.org/10.1016/S0022-2143(99)90157-9
  48. Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, et al. Predictors of outcome in Henoch-Schonlein nephritis in children and adults. Am J Kidney Dis 2006;47: 993-1003 https://doi.org/10.1053/j.ajkd.2006.02.178
  49. Chan JC, Trachtman H. Modulating the progression in IgA nephropathy. Nephron Clin Pract 2006;104:c61-8. https://doi.org/10.1159/000093672
  50. Camacho Diaz JA, Gimenez Llort A, Garcia Garcia L, Jimenez Gonzalez R. Long-term effect of angiotensin-converting inhibitors in children with proteinuria. An Esp Pediatr 2001;55:219-24. https://doi.org/10.1016/S1695-4033(01)77669-2
  51. Dixit MP, Dixit NM, Scott K. Managing Henoch-Schonlein purpura in children with fish oil and ACE inhibitor therapy. Nephrology (Carlton) 2004;9:381-6. https://doi.org/10.1111/j.1440-1797.2004.00320.x
  52. Shin JI, Park JM, Kim JH, Lee JS, Jeong HJ. Methylprednisolone pulse therapy by the Tune-Mendoza protocol in a child with severe Henoch-Schonlein nephritis. Scand J Rheumatol 2006;35:162-3. https://doi.org/10.1080/03009740500304361

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