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Abstract: SA-PO425

C3 Glomerulopathy: Clinical Determinants of Prognosis and Response to Immunosuppression

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Caravaca-Fontan, Fernando, Hospital Universitario 12 de Octubre, Madrid, Spain
  • Cavero escribano, Teresa, Hospital Universitario 12 de Octubre, Madrid, Spain
  • Goicoechea de jorge, Elena, Complutense University Madrid, Madrid, Spain
  • Praga, Manuel, Hospital Universitario 12 de Octubre, Madrid, Spain

Group or Team Name

  • on behalf of GLOSEN group
Background

C3 glomerulopathy (C3G) is a clinicopathological entity secondary to a dysregulation of the alternative complement pathway. Despite significant advances in our understanding of the pathophysiology of this disease, less is known about its prognostic determinants.

Methods

Retrospective, observational study in 28 hospitals belonging to GLOSEN group. All patients fulfilling diagnostic criteria of C3G were included. Clinical, biochemical and histologic parameters of prognostic interest were recorded to analyze the main determinants of disease progression and response to different therapeutic regimens.

Results

The study group consisted of 104 patients: 90 C3 glomerulonephritis (C3GN) and 14 dense deposit disease (DDD).
17% were diagnosed during pediatric age, 49% between 18-50 years, and 34% above 50 years. A monoclonal gammopathy of unknown significance was observed in 16% of patients.
The most common presentation in pediatric age was nephrotic syndrome, whereas acute kidney injury and isolated urinary abnormalities were the most frequent clinical findings in adults. Membranoproliferative glomerulonephritis was the most predominant pattern of injury, with C3 only deposition in 49% of cases.
In a median follow-up of 48 months [IQR:18–100], 45 patients (43%) achieved remission (partial or complete). Treatment with steroids and mycophenolate mofetil was associated with significant improvement in renal survival compared to other treatments or conservative management.
During the follow-up period, 45 patients (43%) developed end-stage renal disease (ESRD). By Cox regression analysis, the main determinants of ESRD were: age (HR: 1.026;C.I.95%: 1.008–1.044; p=0.004), serum creatinine at diagnosis (HR: 1.213;C.I.95%: 1.115–1.318;p<0.0001), and degree of interstitial fibrosis and tubular atrophy (HR: 1.891;C.I.95%: 1.384–2.582;p<0.0001). No differences were found between C3GN and DDD.
Twenty-five patients (24%) underwent kidney transplantation, 14 of which (56%) had disease recurrence after a median follow-up of 12 months [3–107].

Conclusion

C3G is associated with poor renal outcome in a substantial percentage of patients. Steroids and mycophenolate were shown to be the most effective regimen to achieve remission. Older age, elevated serum creatinine and tubular atrophy/interstitial fibrosis were the main determinants of renal survival.