Abstract
This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median estimated glomerular filtration rate (eGFR) was 91.3 ml/min per 1.73 m2, with the median urine protein creatinine ratio (UP:UC) 556 mg/mmol. The treatment protocol comprised daily steroids and cyclophosphamide for 8–12 weeks followed by azathioprine and a reducing regimen of alternate-day steroids for 8–12 months. After a mean follow-up period of 7 years following presentation, 37% made a complete recovery, 40.7% had persistent proteinuria, 7.4% had persistent proteinuria and were on antihypertensive therapy and 14.8% had progressed to end-stage kidney failure (ESKF). Children with poor outcome were older at presentation (p 0.005), had more crescents (p 0.015) and had heavier proteinuria 6 months post initial biopsy (p 0.023). All of the four children with ESKF had nephrotic range proteinuria and greater than 50% crescents on initial biopsy. Despite long-term immunosuppression, the majority of children with HSN grade 3b or higher will have persistent renal abnormalities on long-term follow-up.
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References
Coppo R, Amore A (2004) Henoch-Schönlein purpura. In: Avner ED, Harmon WE, Niaudet P (eds) Textbook of pediatric nephrology, 5th edn. Lippincottt Williams & Wilkins, pp 851–863
Narchi H (2005) Risk of long-term renal impairment and duration of follow-up recommended for Henoch-Schönlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 90:916–920
Meadow SR, Glasgow EF, White RHR, Moncrief MW, Cameron JS, Ogg CS (1972) Schönlein-Henoch nephritis. Q J Med 41:241–258
Koskimies O, Mir S, Rapola J, Vilska J (1981) Henoch-Schonlein nephritis: long-term prognosis of unselected patients. Arch Dis Child 56:482–484
Counahan R, Winterborn MH, White RH, Heaton JM, Meadow SR, Bluett NH, Swetschin H, Cameron JS, Chantler C (1977) Prognosis of Henoch-Schonlein nephritis in children. BMJ 2:11–14
Stewart M, Savage JM, Bell B, McCord B (1988) Long-term renal prognosis of Henoch-Schonlein purpura in an unselected childhood population. Eur J Pediatr 147:113–115
Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena FP (1997) Long-term prognosis of Henoch-Schonlein nephritis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schonlein Purpura. Nephrol Dial Transplant 12:2277–2283
NAPRTCS annual report (2005) Available at https://doi.org/web.emmes.com/study/ped/annlrept/annlrept2005.pdf
UK Renal Registry Report (2005) UK Renal Registry, Bristol UK
Ronkainen J, Ala-Houhala M, Huttunen N-P, Jahnukainen T, Koskimies O, Ormala T, Nuutinen M (2003) Outcome of Henoch-Schönlein nephritis with nephrotic-range proteinuria. Clin Nephrol 60:80–84
Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch nephritis. Pediatr Nephrol 12:238–243
Bunchman TE, Mauer SM, Sibley RK, Vernier RL (1988) Anaphylactoid purpura: characteristics of 16 patients who progressed to renal failure. Pediatr Nephrol 2:393–397
Goldstein AR, White RHR, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 339:280–282
Bergstein J, Leiser J, Andreoli SP (1998) Response of crescentic HSP nephritis to corticosteroid and azathioprine therapy. Clin Nephrol 49:9–14
Ronkainen J, Autio-Harmainen H, Nuutinen M (2003) Cyclosporin A for the treatment of severe Henoch-Schönlein glomerulonephritis. Pediatr Nephrol 18:1138–1142
Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB (2001) Treatment of Henoch-Schönlein purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol 21:128–133
Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M (1999) Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. Am J Kidney Dis 33:427–433
Shenoy M, Ognjanovic M, Coulthard MG (2007) Treating severe Henoch-Schonlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol 122:1167–1171
Tarshish P, Bernstein J, Edelmann CM (2004) Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19:51–56
Schwartz GJ, Haycock GB, Edelman CM, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 51:875–878
Andreoli SP, Bergstein JM (1989) Treatment of severe IgA nephropathy in children. Pediatr Nephrol 3:248–253
Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D (2002) Henoch-Schonlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 13:1271–1278
Kaku Y, Nohara K, Honda S (1998) Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of prognostic factors. Kidney Int 53:1755–1759
Durkan A, Hodson EM, Willis NS, Craig JC (2005) Non-corticosteroid treatment for nephrotic syndrome in children. Cochrane Database Syst Rev Apr 18(2):CD002290
Kawasaki Y, Suzuki J, Suzuki H (2004) Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schonlein nephritis: a clinical and histopathological study. Nephrol Dial Transplant 19:858–864
Foster BJ, Bernard C, Drummond KN, Sharma AK (2000) Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisolone and azathioprine: a clinical and histopathologic study. J Pediatr 136:370–375
Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schönlein purpura. A retrospective cohort study. Lancet 360:666–670
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Shenoy, M., Bradbury, M.G., Lewis, M.A. et al. Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression. Pediatr Nephrol 22, 1717–1722 (2007). https://doi.org/10.1007/s00467-007-0557-4
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DOI: https://doi.org/10.1007/s00467-007-0557-4