Skip to main content
Log in

Etiology and Outcomes of Rapidly Progressive Glomerulonephritis in Children: A Retrospective Cohort Study

  • Research Paper
  • Published:
Indian Pediatrics Aims and scope Submit manuscript

Abstract

Objective

To study the clinico-etiological spectrum and outcomes of children with rapidly progressive glomerulonephritis (RPGN).

Methods

This retrospective cohort study evaluated patients <18 years with RPGN, over an 8-year period (2014–2022), for etiology and kidney outcomes.

Results

Among 68 RPGN cases [median age 10 (7,12) years], 23 (33.8%) had lupus nephritis, 21 (30.9%) C3 glomerulopathy, and 15 (22.1%) infection-related glomerulonephritis (IRGN). At presen-tation, 18 (26.4%) patients had pulmonary edema, 20 (29.4%) had hypertensive emergency and 22 (32.4%) required dialysis. Median (IQR) follow-up duration was 24.5 (12,48) months. The median (IQR) admission eGFR was 19 (10.93, 38.60) mL/min/1.73 m2, which increased to 126 (102.7,142) mL/min/1.73m2 at the last follow-up. At the last follow-up, 39 (57.3%) and 13 (19.1%) patients attained complete and partial renal recovery, respectively; while 16 (23.5%) progressed to CKD stage 2 and beyond. The prevalence of end stage kidney disease (ESKD) was 7.3% at 1-year and 7.7% at the last follow-up. Factors predicting kidney survival were duration of symptoms prior to presentation ≥7 days, crescents ≥37.5%, and presence of fibrous crescents/segmental sclerosis.

Conclusion

Lupus nephritis, was the commonest etiology of RPGN in children. Renal outcomes were determined by pre-admission symptoms, and percentage and stage of crescents.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

References

  1. Trimarchi H. Crescents in primary glomerulonephritis: a pattern of injury with dissimilar actors. A pathophysiologic perspective. Pediatr Nephrol. 2022;37:1205–14.

    Article  PubMed  Google Scholar 

  2. Maliakkal JG, Hicks MJ, Michael M, et al. Renal survival in children with glomerulonephritis with crescents: A pediatric nephrology research consortium cohort study. J Clin Med. 2020;9:2385.

    Article  PubMed  PubMed Central  Google Scholar 

  3. The Southwest Pediatric Nephrology Study Group. A clinicopathologic study of crescentic glomerulonephritis in 50 children. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int. 1985;27:450–58.

    Article  Google Scholar 

  4. Mayer U, Schmitz J, Brasen JH, Pape L. Crescentic glomerulonephritis in children. Pediatr. Nephrol. 2020;35: 829–42.

    Google Scholar 

  5. Jardim HM, Leake J, Risdon RA, Barratt TM, Dillon MJ. Crescentic glomerulonephritis in children. Pediatr. Nephrol. 1992;6:231–35.

    CAS  Google Scholar 

  6. Srivastava RN, Moudgil A, Bagga A, et al. Crescentic glomerulonephritis in children: A review of 43 cases. Am. J. Nephrol. 1992;12:155–61.

    CAS  Google Scholar 

  7. Sinha A, Puri K, Hari P, et al. Etiology and outcome of crescentic glomerulonephritis. Indian Pediatr. 2013;50:283–88.

    Article  PubMed  Google Scholar 

  8. Dewan D, Gulati S, Sharma RK, et al. Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries. Pediatr Nephrol. 2008;23:389–94.

    Article  PubMed  Google Scholar 

  9. Ozlu SG, Caltik A, Aydog O, et al. Crescentic glomerulonephritis in children: A single centre experience. World J Pediatr. 2016;12:225–30.

    Article  PubMed  Google Scholar 

  10. Koyama A, Yamagata K, Makino H, et al. A nationwide survey of rapidly progressive glomerulonephritis in Japan: Etiology, prognosis and treatment diversity. Clin. Exp. Nephrol. 2009;13:633–50.

    Google Scholar 

  11. Rampelli SK, Rajesh NG, Srinivas BH, et al. Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience. Indian J Nephrol. 2016;26:252–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Gupta R, Singh L, Sharma A, et al. Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases. Indian J Pathol Microbiol. 2011;54:497–500.

    Article  PubMed  Google Scholar 

  13. Alsaad, K, Oudah N, Al Ameer A, et al. Glomerulonephritis with crescents in children: Etiology and predictors of renal outcome. ISRN Pediatr. 2011;2011:507298.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Vijayakumar M. Acute and crescentic glomerulonephritis. Indian J Pediatr. 2002;69:1071–75.

    Article  CAS  PubMed  Google Scholar 

  15. Sinha A, Bagga A, Banerjee S, et al. Expert Group of Indian Society of Pediatric Nephrology. Steroid Sensitive Nephrotic Syndrome: Revised Guidelines. Indian Pediatr. 2021;58: 461–81.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Flynn JT, Kaelber DC, Baker-Smith CM, et al. Subcommittee on Screening and Management of High Blood Pressure in Children. Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents. Pediatrics. 2017;140:e20171904.

    Article  PubMed  Google Scholar 

  17. Schwartz GJ, Haycock GB, Edelmann CM, Spitzer A. A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics. 1976;58: 259–63.

    Article  CAS  PubMed  Google Scholar 

  18. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney Int Suppl. 2012;2: 1–138.

    Google Scholar 

  19. Haas M, Seshan SV, Barisoni L, et al. Consensus Definitions for Glomerular Lesions by Light and Electron Microscopy: Recommendations From a Working Group of the Renal Pathology Society. Kidney Int. 2020;98:1120–34.

    Article  PubMed  Google Scholar 

  20. Bajema IM, Wilhelmus S, Alpers CE, et al. Revision of the International Society of Nephrology/Renal Pathology Society Classification for Lupus Nephritis: Clarification of Definitions, and Modified National Institutes of Health Activity and Chronicity Indices. Kidney Int. 2018;93:789–96.

    Article  PubMed  Google Scholar 

  21. Weening JJ, D’Agati VD, Schwartz MM, et al. International Society of Nephrology Working Group on the Classification of Lupus Nephritis; Renal Pathology Society Working Group on the Classification of Lupus Nephritis. The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. Kidney Int. 2004;65:521–30.

    Article  PubMed  Google Scholar 

  22. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–41.

    Article  CAS  PubMed  Google Scholar 

  23. Kidney Disease: Improving Global Outcomes (KDIGO). KDIGO Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;S115–S193.

  24. Wyatt RJ, Julian BA. IgA nephropathy. N Engl J Med. 2013; 368:2402–14

    Article  CAS  PubMed  Google Scholar 

  25. Leung AKC, Barankin B, Leong KF. Henoch-Schönlein purpura in children: An updated review. Curr Pediatr Rev. 2020;16:265–76.

    Article  PubMed  Google Scholar 

  26. Pickering MC, D’Agati VD, Nester CM, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84:1079–89.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Gunasekaran K, Krishnamurthy S, Mahadevan S, et al. Clinical characteristics and outcome of post-infectious glomerulonephritis in children in southern India: A prospective study. Indian J Pediatr. 2015;82:896–903.

    Article  PubMed  Google Scholar 

  28. Patel P, de Guzman M, Hicks MJ, et al. Utility of the 2018 revised ISN/RPS thresholds for glomerular crescents in childhood-onset lupus nephritis: A Pediatric Nephrology Research Consortium study. Pediatr Nephrol. 2022;37: 3139–45.

    Article  PubMed  Google Scholar 

Download references

Funding

Funding: None

Author information

Authors and Affiliations

Authors

Contributions

Contributors: BD, SK, AM, PK, SuK: managed the patients. BD conceptualized the study design and participated in study protocol preparations, recruited patients, participated in data analysis, and drafted the first version of the manuscript. SK conceptualized the study design and critically revised the manuscript. RNG interpreted the histopathology findings. SG helped in statistical analysis. All authors contributed to drafting of the manuscript and approved the final version.

Corresponding author

Correspondence to Sriram Krishnamurthy.

Ethics declarations

Ethics approval: Approved by the Ethics Committee of the institute; No. JIP/IEC/2022/0117 dated May 11, 2022.

Competing interests: None stated.

Electronic supplementary material

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Deepthi, B., Krishnamurthy, S., Ganesh, R.N. et al. Etiology and Outcomes of Rapidly Progressive Glomerulonephritis in Children: A Retrospective Cohort Study. Indian Pediatr 60, 816–821 (2023). https://doi.org/10.1007/s13312-023-3011-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13312-023-3011-1

Keywords

Navigation