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Abstract: SA-PO643

Outcomes by Etiology in Pediatric Glomerulonephritis with Crescents

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Gor, Juhi A., Brody School of Medicine at East Carolina University, Greenville, North Carolina, United States
  • Flynn, Joseph T., Seattle Children's Hospital, Seattle, Washington, United States
  • Pan, Cynthia G., Children's Corporate Center, Milwaukee, Wisconsin, United States
  • Selewski, David T., Medical University of South Carolina, Mount Pleasant, South Carolina, United States
  • Rheault, Michelle N., University of Minnesota, Minneapolis, Minnesota, United States
  • Twombley, Katherine, Medical University of South Carolina, Mount Pleasant, South Carolina, United States
  • Reyes, Loretta, Emory University School of Medicine, Atlanta, Georgia, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Wenderfer, Scott E., Baylor College of Medicine, Houston, Texas, United States
  • Hidalgo, Guillermo, Eastern Carolina University, Greenville, North Carolina, United States
Background

Etiologies of Glomerulonephritis (GN) with crescents in Pediatrics differ from those in adults. Many of the typical etiologies can be categorized into diseases with or without involvement of immune complex (IC) deposition leading to damage and inflammation of glomeruli. Our previous work had identified a composite risk index, involving 4 disease markers: % crescents, presence of fibrous crescents, HTN, and eGFR at biopsy. However, it is unknown whether these 4 markers predict 1 year outcomes in specific classes of glomerulonephritis.

Methods

We reviewed the Pediatric GN with crescents registry which is a multicenter, retrospective (2004-2016) review of subjects < 21 y, with GN with crescents, followed for at least 12 mo. across the US as part of the Midwest Pediatric Nephrology Consortium. Crescentic GN was defined as > 1 crescent per core biopsy according to local pathologist read. Primary outcome of interest was end stage kidney disease (ESKD) at 1 year.

Results

We reviewed records on 305 patients, mean age 11 y, 58% female. There were 53 with pauci immune GN (17%) and 252 with IC GN (82%). 21% of patients in the pauci-immune group reached ESKD at 1 year, as compared to 9.5% in the immune complex group (p-value 0.031). On bivariate analysis in the pauci immune group, variables at time of biopsy associated with ESKD at 1 y. were estimated glomerular filtration rate (eGFR) <15 ml/min/1.73m2 (58 vs. 10% p= 0.001), hypertension (44 vs. 8.6%, p= 0.006), and > 43% crescents (39 vs. 6.7%, p=0.006). Percentage drop in eGFR at 1 year was also higher in the pauci-immune group (20 vs. 10.7%), as compared to the immune complex group, but these results were not statistically significant. In the immune complex group, variables at time of biopsy associated with ESKD at 1 y. were estimated glomerular filtration rate (eGFR) below 15 ml/min/1.73m2 (59 vs. 5.1% p< 0.001), presence of fibrous crescents (16 vs. 6.9% p = 0.034), hypertension (17 vs 3.1% p < 0.001), > 43% crescents (33 vs. 3.9%, p<0.001), and presence of generalized edema (17 vs 5.7% p=0.007

Conclusion

Pauci-immune GN, which constituted only 17% of our cohort, was associated with a significant risk of ESKD at 1 year. Different risk factors at time of biopsy are associated with ESKD at 1 year in pauci immune GN compared to IC GN.