Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis: a nationwide study

  • Mikael KoskelaEmail author
  • Timo Jahnukainen
  • Kira Endén
  • Pekka Arikoski
  • Janne Kataja
  • Matti Nuutinen
  • Elisa Ylinen
Original Article



Optimal treatment of Henoch-Schönlein purpura nephritis (HSN) remains unclear. We evaluated outcome of pediatric HSN patients treated initially with either methylprednisolone (MP) or cyclosporine A (CyA) in Finland between 1996 and 2011.


Outcome of 62 HSN patients was evaluated by screening urine and blood samples (n = 51) or by collecting clinical parameters from medical charts until last follow-up visit (n = 11). Sixty (97%) patients had nephrotic-range proteinuria and/or ISKDC grade ≥ III before initial treatment. Patients were initially treated with either MP pulses (n = 42) followed by oral prednisone or with CyA (n = 20). Fifty-nine (95%) patients received angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers.


Mean follow-up time was 10.8 years (range 3.2–21.2 years). One patient developed end-stage renal disease and another had decreased renal function (eGFR < 60 mL/min/1.73m2), both initially treated with MP (3%). Six patients (5 MP, 1 CyA) had eGFR between 60 and 89 mL/min/1.73m2 (10%). Eighteen patients (13 MP, 5 CyA) had proteinuria and/or hematuria (29%) and four of them had proteinuria > 0.5 g/day at end of follow-up. Sixteen (38%) MP-treated and two (10%) CyA-treated patients needed additional immunosuppressive treatment (RR 3.81, 95% CI 1.16–14.3, p = 0.035). Late initiation of treatment was associated with an increased risk for persistent proteinuria.


Long-term outcome was relatively good in both treatment groups. However, since urinary abnormalities may persist or develop, long-term follow-up of HSN patients is mandatory. Early initiation of treatment had a favorable effect on proteinuria.


Outcome Immunosuppression Angiotensin-converting enzyme inhibitor Vasculitis IgA glomerulonephritis Nephrotic syndrome Children 



We would like to thank Mrs. Anna Vuolteenaho for language editing.


This work was supported by a grant to M.K. from the Finnish Kidney Foundation, the Päivikki and Sakari Sohlberg Foundation, the Foundation for Paediatric Research, the Paulo Foundation, and the Alma and K.A. Snellman Foundation, Oulu, Finland.

Compliance with ethical standards

Informed consent was obtained from all patients (and their guardians if necessary) who participated in the follow-up screening. The study was approved by the ethics committee of Helsinki University Hospital. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

467_2019_4238_MOESM1_ESM.pdf (201 kb)
Supplementary Table S1 (PDF 201 kb)


  1. 1.
    Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of henoch-schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197–1202CrossRefGoogle Scholar
  2. 2.
    Saulsbury FT (2007) Clinical update: Henoch-schonlein purpura. Lancet 369:976–978CrossRefGoogle Scholar
  3. 3.
    Narchi H (2005) 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 90:916–920CrossRefGoogle Scholar
  4. 4.
    Koskimies O, Mir S, Rapola J, Vilska J (1981) Henoch-schonlein nephritis: long-term prognosis of unselected patients. Arch Dis Child 56:482–484CrossRefGoogle Scholar
  5. 5.
    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 147:113–115CrossRefGoogle Scholar
  6. 6.
    Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schonlein nephritis. Lancet 339:280–282CrossRefGoogle Scholar
  7. 7.
    Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet 360:666–670CrossRefGoogle Scholar
  8. 8.
    Hahn D, Hodson EM, Willis NS, Craig JC (2015) Interventions for preventing and treating kidney disease in Henoch-Schonlein purpura (HSP). Cochrane Database Syst Rev 8:CD005128Google Scholar
  9. 9.
    Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243CrossRefGoogle Scholar
  10. 10.
    Kawasaki Y, Suzuki J, Suzuki H (2004) 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 19:858–864CrossRefGoogle Scholar
  11. 11.
    Ronkainen J, Autio-Harmainen H, Nuutinen M (2003) Cyclosporin A for the treatment of severe Henoch-Schonlein glomerulonephritis. Pediatr Nephrol 18:1138–1142CrossRefGoogle Scholar
  12. 12.
    Shin JI, Park JM, Shin YH, Kim JH, Kim PK, Lee JS, Jeong HJ (2005) Cyclosporin A therapy for severe Henoch-Schonlein nephritis with nephrotic syndrome. Pediatr Nephrol 20:1093–1097CrossRefGoogle Scholar
  13. 13.
    Park JM, Won SC, Shin JI, Yim H, Pai KS (2011) Cyclosporin A therapy for Henoch-Schonlein nephritis with nephrotic-range proteinuria. Pediatr Nephrol 26:411–417CrossRefGoogle Scholar
  14. 14.
    Jauhola O, Ronkainen J, Autio-Harmainen H, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Nuutinen M (2011) Cyclosporine A vs. methylprednisolone for Henoch-Schonlein nephritis: a randomized trial. Pediatr Nephrol 26:2159–2166CrossRefGoogle Scholar
  15. 15.
    Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, Ormala T, Turtinen J, Nuutinen M (2006) Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr 149:241–247CrossRefGoogle Scholar
  16. 16.
    Kidney disease: Improving global outcomes (KDIGO) CKD work group (2013) KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl 3:1–150CrossRefGoogle Scholar
  17. 17.
    Whelton PK, Carey RM (2018) The 2017 American College of Cardiology/American Heart Association clinical practice guideline for high blood pressure in adults. JAMA Cardiol 3:352–353CrossRefGoogle Scholar
  18. 18.
    Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, de Ferranti SD, Dionne JM, Falkner B, Flinn SK, Gidding SS, Goodwin C, Leu MG, Powers ME, Rea C, Samuels J, Simasek M, Thaker VV, Urbina EM, SUBCOMMITTEE ON SCREENING AND MANAGEMENT OF HIGH BLOOD PRESSURE IN CHILDREN (2017) Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 140:e20171904. CrossRefGoogle Scholar
  19. 19.
    Schwartz GJ, Munoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, Furth SL (2009) New equations to estimate GFR in children with CKD. J Am Soc Nephrol 20:629–637CrossRefGoogle Scholar
  20. 20.
    Levey AS, Stevens LA, Schmid CH, Zhang YL, Castro AF 3rd, Feldman HI, Kusek JW, Eggers P, Van Lente F, Greene T, Coresh J, CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) (2009) A new equation to estimate glomerular filtration rate. Ann Intern Med 150:604–612CrossRefGoogle Scholar
  21. 21.
    Vittinghoff E, McCulloch CE (2007) Relaxing the rule of ten events per variable in logistic and Cox regression. Am J Epidemiol 165:710–718CrossRefGoogle Scholar
  22. 22.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Turtinen J, Nuutinen M (2010) Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 95:877–882CrossRefGoogle Scholar
  23. 23.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Nuutinen M (2012) Outcome of Henoch-Schonlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol 27:933–939CrossRefGoogle Scholar
  24. 24.
    Wakaki H, Ishikura K, Hataya H, Hamasaki Y, Sakai T, Yata N, Kaneko T, Honda M (2011) Henoch-Schonlein purpura nephritis with nephrotic state in children: predictors of poor outcomes. Pediatr Nephrol 26:921–925CrossRefGoogle Scholar
  25. 25.
    Scharer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F (1999) Clinical outcome of Schonlein-Henoch purpura nephritis in children. Pediatr Nephrol 13:816–823CrossRefGoogle Scholar
  26. 26.
    Hennies I, Gimpel C, Gellermann J, Moller K, Mayer B, Dittrich K, Buscher AK, Hansen M, Aulbert W, Wuhl E, Nissel R, Schalk G, Weber LT, Pohl M, Wygoda S, Beetz R, Klaus G, Fehrenbach H, Konig S, Staude H, Beringer O, Bald M, Walden U, von Schnakenburg C, Bertram G, Wallot M, Haffner K, Wiech T, Hoyer PF, Pohl M, German Society of Pediatric Nephrology (2018) Presentation of pediatric Henoch-Schonlein purpura nephritis changes with age and renal histology depends on biopsy timing. Pediatr Nephrol 33:277–286CrossRefGoogle Scholar
  27. 27.
    Shin JI, Park JM, Kim JH, Lee JS, Jeong HJ (2006) Factors affecting histological regression of crescentic Henoch-Schonlein nephritis in children. Pediatr Nephrol 21:54–59CrossRefGoogle Scholar
  28. 28.
    Ninchoji T, Kaito H, Nozu K, Hashimura Y, Kanda K, Kamioka I, Shima Y, Hamahira K, Nakanishi K, Tanaka R, Yoshikawa N, Iijima K, Matsuo M (2011) Treatment strategies for Henoch-Schonlein purpura nephritis by histological and clinical severity. Pediatr Nephrol 26:563–569CrossRefGoogle Scholar
  29. 29.
    Coppo R, Peruzzi L, Amore A, Piccoli A, Cochat P, Stone R, Kirschstein M, Linne T (2007) 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 18:1880–1888CrossRefGoogle Scholar
  30. 30.
    Fretzayas A, Sionti I, Moustaki M, Papadimitriou A, Nicolaidou P (2008) Henoch-Schonlein purpura: a long-term prospective study in Greek children. J Clin Rheumatol 14:324–331CrossRefGoogle Scholar
  31. 31.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Nuutinen M (2010) Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study. Arch Dis Child 95:871–876CrossRefGoogle Scholar
  32. 32.
    Prais D, Amir J, Nussinovitch M (2007) Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol 13:25–28CrossRefGoogle Scholar

Copyright information

© IPNA 2019

Authors and Affiliations

  • Mikael Koskela
    • 1
    • 2
    Email author
  • Timo Jahnukainen
    • 2
  • Kira Endén
    • 3
  • Pekka Arikoski
    • 4
  • Janne Kataja
    • 5
  • Matti Nuutinen
    • 6
    • 7
  • Elisa Ylinen
    • 2
  1. 1.Children’s Hospital, Pediatric Research Center, Helsinki University HospitalUniversity of HelsinkiHelsinkiFinland
  2. 2.Department of Pediatric Nephrology and Transplantation, New Children’s HospitalUniversity of Helsinki and Helsinki University HospitalHelsinkiFinland
  3. 3.Department of PediatricsTampere University HospitalTampereFinland
  4. 4.Department of PediatricsKuopio University Hospital and University of Eastern FinlandKuopioFinland
  5. 5.Pediatric and Adolescent MedicineTurku University HospitalTurkuFinland
  6. 6.Department of Children and AdolescentsOulu University HospitalOuluFinland
  7. 7.PEDEGO Research Unit, Research Unit for Pediatrics, Dermatology, Clinical Genetics, Obstetrics and GynecologyMedical Research Center Oulu (MRC Oulu)OuluFinland

Personalised recommendations