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Abstract: FR-OR087

Validation of C3 Glomerulopathy Histopathologic Index in a Large Multicenter Cohort Study

Session Information

  • Lupus and Then Some
    November 08, 2019 | Location: Ballroom C, Walter E. Washington Convention Center
    Abstract Time: 05:42 PM - 05:54 PM

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
  • Lucientes, Laura, Universidad Complutense de Madrid, 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 Histopathologic Index (C3G-HI) has been recently proposed as a tool for assessing both activity and chronicity of kidney biopsies in patients affected with C3 glomerulopathy (C3G).
The aim of this study was to evaluate the utility and reproducibility of C3G-HI for predicting renal prognosis in C3G.

Methods

Multicenter, retrospective cohort study in 37 nephrology departments belonging to GLOSEN group.
All patients fulfilling diagnostic criteria of C3G were included. Kidney biopsies were evaluated by each participating center, and lesions were scored according to the C3G-HI. Demographic, clinical and biochemical parameters of prognostic interest were recorded and used to analyze the main determinants of disease progression and response to different therapeutic regimens.

Results

The study group consisted of 134 patients: 114 C3 glomerulonephritis (C3GN) and 20 dense deposit disease (DDD).
Membranoproliferative glomerulonephritis was the most predominant pattern of injury. No significant differences were observed in the parameters of activity across age groups, except for interstitial inflammation that was greater in older patients. However, pediatric patients had a significant lower degree of glomerulosclerosis, tubular atrophy and interstitial fibrosis. No significant clinicopathological differences were observed between C3GN and DDD patients.
During a median follow-up of 43 months, 53 patients (40%) developed end-stage renal disease (ESRD). Renal survival was significantly worse in patients in the lower tertiles of Total Chronicity Score.
By Cox regression analysis, the main determinants of ESRD were: age (hazard ratio [HR]: 1.019; C.I.95%:1.003–1.034; p=0.016), serum creatinine at baseline (HR:1.131; C.I.95%:1.037–1.235; p=0.006), proteinuria at baseline (HR:1.076; C.I.95%:1.007–1.151; p=0.031), Total Chronicity Score (HR:1.326;C.I.95%:1.118–1.480;p<0.0001) and therapy with mycophenolate mofetil plus steroids (HR: 0.344; C.I.95%: 0.150–0.787; p=0.012).

Conclusion

C3G-HI provides useful predictive information in C3G, being chronicity parameters the main pathologic determinants of renal prognosis. Older age, elevated serum creatinine and proteinuria are the main clinical determinants of renal survival, whereas treatment with steroids and mycophenolate mofetil was associated with better outcome.