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Abstract: PO1554

C3 Glomerulopathy in Children and Adolescents

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Borovitz, Yael, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
  • Alfandary, Hadas, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
  • Dagan, Amit, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
  • Haskin, Orly, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
  • Landau, Daniel, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
  • Davidovits, Miriam, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
Background

C3 glomerulopathy (C3G) has been classified as a glomerular complement mediated disease with predominant C3 deposits almost a decade ago. Data regarding clinical course, treatment options and long term prognosis in children and adolescents is still scarce.
The aim of the study was to retrospectively describe a single tertiary center's experience with C3G in the pediatric population, and correlate presentation, pathology, complement findings, response to treatment and disease prognosis.

Methods

A retrospective cohort study. Patients presented with C3G by the age of 18 years compromised the study group. All cases underwent kidney biopsy at presentation. Repeated kidney biopsy was performed on a need basis, in native or transplanted kidneys. Definition of C3G was based on the 2013 consensus guidelines. Patients underwent complement workup and genetic tests. Treatment regimen was not uniform.

Results

17 patients were diagnosed with C3 glomerulopathy. Features of Dense deposit disease (DDD) were found in 8 patients, C3 glomerulonephritis in 6 patients. For 3 patients EM was not available. Mean age at diagnosis was 12.7 years (range 1.9 – 17.3). 6 girls and 11 boys. Median follow up 4.4 years (range 1.1-20.9).
Treatment modalities ranged from ACE inhibitors and Angiotensin receptor blockers to corticosteroids, Mycophenolate Mofetil, Plasmapheresis, Rituximab and Eculizumab.
Only 2 (12%) patients achieved complete remission. 4 (23%) patients reached end stage renal failure and had kidney transplantation. All of them had disease recurrence in the transplanted kidney. Complement workup was positive for C3Nef in 6 patients, C4Nef in 2 patients, C5Nef in 2 patients, factor H antibodies in 2 patients. Genetic testing was positive in one patient. Elevated creatinine at presentation, severe proteinuria, DDD in kidney biopsy– were correlated with worse prognosis.

Conclusion

Understanding the pathophysiology of C3G as a complement mediated disease has progressed during the past years. Still no guidelines exist regarding treatment and prognosis in the pediatric population. Our cohort presented a wide variability in disease course and presentation. Further understanding of the correlation between exact complement abnormality and C3G prognosis is warranted, especially now when new complement system blockers may become available.