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C3 glomerulonephritis with a severe crescentic phenotype.

Aishwarya Ravindran1, Fernando C Fervenza2, Richard J H Smith3

  • 1Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Pediatric Nephrology (Berlin, Germany)
|June 9, 2017
PubMed
Summary

C3 glomerulopathy (C3G) can present as severe crescentic glomerulonephritis in young patients. Early recognition and treatment are crucial for managing this rare condition.

Keywords:
C3 glomerulonephritisC3 glomerulopathyCrescentsDense deposit diseaseKidney

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Area of Science:

  • Nephrology
  • Complement System Biology
  • Glomerular Diseases

Background:

  • C3 glomerulopathy (C3G) is a rare kidney disease caused by complement dysregulation.
  • It is classified into C3GN and DDD based on ultrastructural findings.
  • C3GN typically shows membranoproliferative glomerulonephritis, but a subset exhibits severe necrotizing and crescentic features.

Purpose of the Study:

  • To describe the clinical, pathological, and treatment outcomes of C3GN patients with severe crescentic glomerulonephritis.
  • To compare these findings with similar cases of dense deposit disease (DDD).

Main Methods:

  • Case series presentation of four C3GN patients with severe crescentic glomerulonephritis.
  • Comparison with four DDD patients exhibiting similar severe pathology.
  • Analysis of clinical features, kidney biopsy findings, complement evaluation, treatment, and follow-up.

Main Results:

  • Young patients presented with hematuria and proteinuria.
  • Kidney biopsies showed numerous crescents and bright glomerular C3 staining.
  • Three of four C3GN patients and two of four DDD patients maintained stable renal function post-treatment with immunosuppressants.

Conclusions:

  • C3G with a severe crescentic phenotype is rare, affecting young individuals.
  • Treatment response to steroids and immunosuppressants is variable.
  • Recognizing this entity is vital for appropriate management of crescentic glomerulonephritis.