Skip to main content

Advertisement

SpringerLink
Log in

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

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Background

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.

Methods

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.

Results

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.

Conclusions

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.

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

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  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 360:1197–1202

    Article  PubMed  Google Scholar 

  2. Saulsbury FT (2007) Clinical update: Henoch-schonlein purpura. Lancet 369:976–978

    Article  PubMed  Google Scholar 

  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 90:916–920

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Koskimies O, Mir S, Rapola J, Vilska J (1981) Henoch-schonlein nephritis: long-term prognosis of unselected patients. Arch Dis Child 56:482–484

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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 147:113–115

    Article  CAS  PubMed  Google Scholar 

  6. Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schonlein nephritis. Lancet 339:280–282

    Article  CAS  PubMed  Google Scholar 

  7. Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet 360:666–670

    Article  PubMed  Google Scholar 

  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:CD005128

    Google Scholar 

  9. Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243

    Article  CAS  PubMed  Google Scholar 

  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 19:858–864

    Article  CAS  PubMed  Google Scholar 

  11. Ronkainen J, Autio-Harmainen H, Nuutinen M (2003) Cyclosporin A for the treatment of severe Henoch-Schonlein glomerulonephritis. Pediatr Nephrol 18:1138–1142

    Article  PubMed  Google Scholar 

  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 20:1093–1097

    Article  PubMed  Google Scholar 

  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 26:411–417

    Article  PubMed  Google Scholar 

  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 26:2159–2166

    Article  PubMed  Google Scholar 

  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 149:241–247

    Article  CAS  PubMed  Google Scholar 

  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 3:1–150

    Article  Google Scholar 

  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 3:352–353

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1542/peds.2017-1904

    Article  PubMed  Google Scholar 

  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 20:629–637

    Article  PubMed  PubMed Central  Google Scholar 

  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 150:604–612

    Article  PubMed  PubMed Central  Google Scholar 

  21. Vittinghoff E, McCulloch CE (2007) Relaxing the rule of ten events per variable in logistic and Cox regression. Am J Epidemiol 165:710–718

    Article  PubMed  Google Scholar 

  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 95:877–882

    Article  PubMed  Google Scholar 

  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 27:933–939

    Article  PubMed  Google Scholar 

  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 26:921–925

    Article  PubMed  Google Scholar 

  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 13:816–823

    Article  CAS  PubMed  Google Scholar 

  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 33:277–286

    Article  PubMed  Google Scholar 

  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 21:54–59

    Article  PubMed  Google Scholar 

  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 26:563–569

    Article  PubMed  Google Scholar 

  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 18:1880–1888

    Article  CAS  PubMed  Google Scholar 

  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 14:324–331

    Article  PubMed  Google Scholar 

  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 95:871–876

    Article  PubMed  Google Scholar 

  32. Prais D, Amir J, Nussinovitch M (2007) Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol 13:25–28

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

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

Funding

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.

Author information

Authors and Affiliations

  1. Children’s Hospital, Pediatric Research Center, Helsinki University Hospital, University of Helsinki, Helsinki, Finland

    Mikael Koskela

  2. Department of Pediatric Nephrology and Transplantation, New Children’s Hospital, University of Helsinki and Helsinki University Hospital, Stenbäckinkatu 9, 00290, Helsinki, Finland

    Mikael Koskela, Timo Jahnukainen & Elisa Ylinen

  3. Department of Pediatrics, Tampere University Hospital, Tampere, Finland

    Kira Endén

  4. Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Kuopio, Finland

    Pekka Arikoski

  5. Pediatric and Adolescent Medicine, Turku University Hospital, Turku, Finland

    Janne Kataja

  6. Department of Children and Adolescents, Oulu University Hospital, Oulu, Finland

    Matti Nuutinen

  7. PEDEGO Research Unit, Research Unit for Pediatrics, Dermatology, Clinical Genetics, Obstetrics and Gynecology, Medical Research Center Oulu (MRC Oulu), Oulu, Finland

    Matti Nuutinen

Authors
  1. Mikael Koskela
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Timo Jahnukainen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kira Endén
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Pekka Arikoski
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Janne Kataja
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Matti Nuutinen
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Elisa Ylinen
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mikael Koskela.

Ethics declarations

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.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Koskela, M., Jahnukainen, T., Endén, K. et al. Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis: a nationwide study. Pediatr Nephrol 34, 1447–1456 (2019). https://doi.org/10.1007/s00467-019-04238-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-019-04238-2

Keywords

Search

Navigation