Does MEST-C score predict outcomes in pediatric Henoch-Schönlein purpura nephritis?
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.
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).
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%.
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.
KeywordsOxford Classification ISKDC Chronic kidney disease IgA nephropathy Hypertension Proteinuria Children
Estimated glomerular filtration rate
Chronic kidney disease
Henoch-Schönlein purpura nephritis
The International Study of Kidney Disease in Children
Receiver operator characteristics
Urine protein-to-creatinine ratio
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.
- 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.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
- 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
- 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.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
- 16.Jennette J (2006) Immunoglobin A nephropathy and Henoch-Schonlein purpura. In: Fundamentals of renal pathology. Springer, New York, pp 61–69Google Scholar
- 20.(2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555Google Scholar
- 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
- 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
- 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.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
- 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