Advertisement

Does MEST-C score predict outcomes in pediatric Henoch-Schönlein purpura nephritis?

  • Adam JimenezEmail author
  • Ashton Chen
  • Jen-Jar Lin
  • Andrew M. South
Original Article

Abstract

Background

Children with Henoch-Schönlein purpura nephritis (HSPN) have an increased risk of chronic kidney disease (CKD). Renal biopsy diagnostic of HSPN is graded using the International Study of Kidney Disease in Children criteria, which do not predict outcomes. The 2016 Oxford Classification’s MEST-C scoring system predicts outcomes in adults with histologically identical IgA nephropathy, but evidence of its utility in pediatric HSPN is lacking. Our hypothesis was that MEST-C scores predict outcomes in children with HSPN.

Methods

A retrospective cohort analysis of data from 32 children with HSPN who underwent renal biopsy was performed. We used logistic regression and receiver operator characteristic curves to analyze the ability of MEST-C to predict the composite outcome of hypertension (blood pressure ≥ 95% for age/sex/height), CKD (estimated glomerular filtration rate < 90 mL/min/1.73 m2), or proteinuria (urine protein-to-creatinine ratio > 0.2 mg/mg).

Results

The median age at diagnosis was 7.9 years (IQR 5.8, 11.7); 56% were male, 19% were Hispanic, and 9% were Black. After a median follow-up of 2.7 years, 38% of patients (n = 12) reached the outcome. S1 score was significantly associated with the outcome (OR 7.9, 95% CI 1.5–42.6). S1 accurately predicted the outcome (area under the curve 0.72, 95% CI 0.55–0.88) with 58.3% sensitivity and 85.0% specificity, indicating a positive predictive value of 70.0% and a negative predictive value of 77.3%.

Conclusions

S1 accurately predicted our composite outcome of hypertension, CKD, and proteinuria in a diverse cohort of U.S. children with HSPN. Further investigation is warranted to validate these findings.

Keywords

Oxford Classification ISKDC Chronic kidney disease IgA nephropathy Hypertension Proteinuria Children 

Abbreviations

eGFR

Estimated glomerular filtration rate

CKD

Chronic kidney disease

HSPN

Henoch-Schönlein purpura nephritis

IQR

Interquartile range

ISKDC

The International Study of Kidney Disease in Children

IgAN

IgA nephropathy

ROC

Receiver operator characteristics

UPCR

Urine protein-to-creatinine ratio

Notes

Acknowledgments

We would like to thank Dan P. Goldstein and Marcia Voigt for their assistance with the collection of the data.

Compliance with ethical standards

The Wake Forest School of Medicine Institutional Review Board approved the study.

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Davin J, Ten Berge I, Weening J (2001) What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis? Kidney Int 59:823–834.  https://doi.org/10.1046/j.1523-1755.2001.059003823.x CrossRefGoogle Scholar
  2. 2.
    Hahn D, Hodson E, Willis N, Craig J (2015) Interventions for preventing and treating kidney disease in Henoch-Schönlein purpura (HSP). Cochrane Database Syst Rev.  https://doi.org/10.1002/14651858.CD005128.pub3
  3. 3.
    Schärer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F (1999) Clinical outcome of Schönlein-Henoch purpura nephritis in children. Pediatr Nephrol 13:816–823.  https://doi.org/10.1007/s004670050707 CrossRefGoogle Scholar
  4. 4.
    Chen J, Mao J (2015) Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr 11:29–34.  https://doi.org/10.1007/s12519-014-0534-5 CrossRefGoogle Scholar
  5. 5.
    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.  https://doi.org/10.1136/adc.2005.074641 CrossRefGoogle Scholar
  6. 6.
    Davin J (2011) Henoch-Schonlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol 6:679–689.  https://doi.org/10.2215/CJN.06710810 CrossRefGoogle Scholar
  7. 7.
    Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, Locatelli F, Cagnoli L (2006) Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis 47:993–1003.  https://doi.org/10.1053/j.ajkd.2006.02.178 CrossRefGoogle Scholar
  8. 8.
    Davin J, Coppo R (2013) Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch–Schönlein purpura nephritis. Pediatr Nephrol 28:1897–1903.  https://doi.org/10.1007/s00467-013-2550-4 CrossRefGoogle Scholar
  9. 9.
    Soylemezoglu O, Ozkaya O, Ozen S, Bakkaloglu A, Dusunel R, Peru H, Donmez O, Buyan N, Mir S, Gur-Guven A, Alpay H, Ekim M, Aksu N, Soylu A, Gok F, Poyrazoglu H, Sonmez F (2009) Henoch-Schonlein nephritis: a nationwide study. Nephron Clin Pract 112:c199–c204.  https://doi.org/10.1159/000218109 CrossRefGoogle Scholar
  10. 10.
    Foster B, Bernard C, Drummond K, Sharma A (2000) Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study. J Pediatr 136:370–375.  https://doi.org/10.1067/mpd.2000.103448 CrossRefGoogle Scholar
  11. 11.
    Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena F (1997) Long-term prognosis of Henoch-Schönlein nephritis in adults and children. Italian Group of Renal Immunopathology collaborative study on Henoch-Schönlein purpura. Nephrol Dial Transplant 12:2277–2283CrossRefGoogle Scholar
  12. 12.
    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.  https://doi.org/10.1016/S0140-6736(02)09835-5 CrossRefGoogle Scholar
  13. 13.
    Trimarchi H, Barratt J, Cattran DC, Cook T, Coppo R, Haas M, Liu Z, Roberts I, Yuzawa Y, Zhang H, Feehally J, Alpers C, Asunis A, Barbour S, Becker J, Ding J, Espino G, Ferrario F, Fogo A, Hladunewich M, Joh K, Katafuchi R, Matsuzaki K, Nakanishi K, Pani A, Perera R, Perkowska A, Reich H, Shima Y, Soares M, Suzuki Y, Takahashi K, Troyanov S, Verhave J, Wang S, Weening J, Wyatt R, Yoshikawa N, Zeng C (2017) Oxford classification of IgA nephropathy 2016: an update from the IgA Nephropathy Classification Working Group. Kidney Int 91:1014–1021.  https://doi.org/10.1016/j.kint.2017.02.003 CrossRefGoogle Scholar
  14. 14.
    Roberts I, Cook H, Troyanov S, Alpers C, Amore A, Baratt J, Berthoux F, Bonsib S, Bruijin J, Cattran D, Coppo R, D’Agati V, Amico G, Emancipator S, Emma F, Feehally J, Ferrario F, Fervenza F, Florquin S, Fogo A, Geddes C, Groene H, Herzenberg A, Hill P, Hogg R, Hsu S, Jennette C, Joh K, Julian B, Kawamura T, Lai F, Li L, Li P, Liu Z, Macjinnon B, Mezzano S, Schena P, Tomino Y, Walker P, Wang H, Weening J, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int 76:546–556.  https://doi.org/10.1038/ki.2009.168 CrossRefGoogle Scholar
  15. 15.
    Magistroni R, D’Agati V, Appel G, Kiryluk K (2015) New developments in the genetics, pathogenesis, and therapy of IgA nephropathy. Kidney Int 88:974–989.  https://doi.org/10.1038/ki.2015.252 CrossRefGoogle Scholar
  16. 16.
    Jennette J (2006) Immunoglobin A nephropathy and Henoch-Schonlein purpura. In: Fundamentals of renal pathology. Springer, New York, pp 61–69Google Scholar
  17. 17.
    Xu K, Zhang L, Ding J, Wang S, Su B, Xiao H, Wang F, Zhong X, Ly Y (2018) Value of the Oxford classification of IgA nephropathy in children with Henoch–Schönlein purpura nephritis. J Nephrol 31:279–286.  https://doi.org/10.1007/s40620-017-0457-z CrossRefGoogle Scholar
  18. 18.
    Szeto C, Choi P, To K, Li P, Hui J, Chow K, Leung K, Lui S, Mac-Moune L (2001) Grading of acute and chronic renal lesions in Henoch-Schönlein purpura. Mod Pathol 14:635–640.  https://doi.org/10.1038/modpathol.3880364 CrossRefGoogle Scholar
  19. 19.
    Pohl M (2015) Henoch–Schönlein purpura nephritis. Pediatr Nephrol 30:245–252.  https://doi.org/10.1007/s00467-014-2815-6 CrossRefGoogle Scholar
  20. 20.
    (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555Google Scholar
  21. 21.
    Ginsberg J, Chang B, Matarese R, Garella S (1983) Use of single voided urine samples to estimate quantitative proteinuria. N Engl J Med 309:1543–1546.  https://doi.org/10.1056/NEJM198312223092503 CrossRefGoogle Scholar
  22. 22.
    Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group. KDIGO (2012) Clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl 3:1–150Google Scholar
  23. 23.
    Inker L, Schmid C, Tighiouart H, Feldmen H, Greene T, Kusek J, Manzi J, Van Lente F, Zhang Y, Coresh J, Levey A (2012) Estimating glomerular filtration rate from serum creatinine and cystatin C. N Engl J Med 367:20–29.  https://doi.org/10.1056/NEJMoa1114248 CrossRefGoogle Scholar
  24. 24.
    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.  https://doi.org/10.1097/01.ASN.0000013883.99976.22 CrossRefGoogle Scholar
  25. 25.
    Edström Halling S, Söderberg M, Berg U (2009) Treatment of severe Henoch–Schönlein and immunoglobulin A nephritis. A single center experience. Pediatr Nephrol 24:91–97.  https://doi.org/10.1007/s00467-008-0990-z CrossRefGoogle Scholar
  26. 26.
    Ho Kim C, Jin Lim B, Sung Bae Y, Eun Kwon Y, Heon Nam K, Sook Park K, Yeong An S, Mo Koo H, Mee Doh F, Jung Lee M, Jung OH, Yoo T, Kang S, Hun Choi K, Joo Jeong H, Han Seung H (2014) Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch–Schönlein purpura nephritis in adults. Mod Pathol 27:972–982.  https://doi.org/10.1038/modpathol.2013.222 CrossRefGoogle Scholar
  27. 27.
    Levey A, Eckardt K, Tsukamoto Y, Levin A, Coresh J, Rossert J, Zeeuw D, Hostetter T, Lameire N, Eknoyan G (2005) Definition and classification of chronic kidney disease: a position statement from Kidney Disease: Improving Global Outcomes (KDIGO). Kidney Int 67:2089–2100.  https://doi.org/10.1111/j.1523-1755.2005.00365.x CrossRefGoogle Scholar
  28. 28.
    Shin J, Park J, Shin Y, Kim J, Lee J, Kim P, Jeong H (2005) Can azathioprine and steroids alter the progression of severe Henoch-Schönlein nephritis in children? Pediatr Nephrol 20:1087–1092.  https://doi.org/10.1007/s00467-005-1869-x CrossRefGoogle Scholar
  29. 29.
    Kanai H, Sawanobori E, Kobayashi A, Matsishita K, Sugita K, Higashida K (2011) Early treatment with methylprednisolone pulse therapy combined with tonsillectomy for heavy proteinuric Henoch-Schönlein purpura nephritis in children. Nephron Extra 1:101–111.  https://doi.org/10.1159/000333010 CrossRefGoogle Scholar
  30. 30.
    Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243CrossRefGoogle Scholar
  31. 31.
    Delbet J, Hogan J, Aoun B, Stoica I, Salomon R, Decramer S, Brocheriou I, Deschenes G, Ulinski T (2017) Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents. Pediatr Nephrol 32:1193–1199.  https://doi.org/10.1007/s00467-017-3604-9 CrossRefGoogle Scholar
  32. 32.
    Tanaka H, Suzuki K, Nakahata T, Ito E, Waga S (2003) Early treatment with oral immunosuppressants in severe proteinuric purpura nephritis. Pediatr Nephrol Berl Ger 18:347–350.  https://doi.org/10.1007/s00467-003-1094-4 Google Scholar
  33. 33.
    Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N (1998) Multiple combined therapy for severe Henoch-Schönlein nephritis in children. Pediatr Nephrol 12:244–248.  https://doi.org/10.1007/s004670050447 CrossRefGoogle Scholar

Copyright information

© IPNA 2019

Authors and Affiliations

  1. 1.Wake Forest School of MedicineWinston-SalemUSA
  2. 2.Section of Nephrology, Department of PediatricsWake Forest School of MedicineWinston-SalemUSA
  3. 3.Department of Epidemiology and Prevention, Division of Public Health SciencesWake Forest School of MedicineWinston-SalemUSA
  4. 4.Cardiovascular Sciences CenterWake Forest School of MedicineWinston-SalemUSA

Personalised recommendations