ESPN 50th Annual Meeting

ESPN 2017


 
Pediatric rapidly progressive glomerulonephritis: 11 years experience
SAMAR ATEF 1 HANAN FATHY 1 MOHAMED THABET 1

1- ALEXANDRIA UNIVERSITY
 
Introduction:

In this study, we aimed to evaluate etiology, clinicopathological features and outcome of rapidly progressive glomerulonephritis (RPGN) in children followed at pediatric nephrology unit, Alexandria University over 11 years (2004-2014).

Material and methods:

We investigated 41 children (22 girls and 19 boys, mean age 8.3 ± 3.3 years) with crescentic glomerulonephritis (CrGN) retrospectively, and compared them to another 25 cases (16 girls, 9 boys) who had RPGN (glomerulonephritis with rapidly progressive renal failure) but with less than 20% crescentic glomeruli in renal biopsy (non-crescentic RPGN).

Results:

Among CrGN group, type II (immune complex) accounted for 78% of cases and type III (pauci-immune) accounted for the other 22% who were chiefly ANCA-negative (88.9%). When comparing crescentic and non-crescentic RPGN, children with CrGN were more hypertensive (P=0.015), more oligo-anuric (P=0.05), they had lower hemoglobin (p=0.002), and were more proteinuric (p=0.027).There was no significant difference between them regarding serum creatinine (p=0.746) nor dialysis dependency at presentation (p=0.152). Glomerular sclerosis (p=0.001), tubular atrophy (p=0.001) and interstitial inflammatory infiltrate (p=0.023) were more frequent in biopsies of crescentic than non-crescentic group. At latest follow-up; 17.3 % of CrGN cohort had passed away, 23.1% had achieved complete remission and around one fifth had developed end-stage kidney disease. Crescentic lupus nephritis showed the highest risk of disease progression. Worst outcome of CrGN was associated elder age (p=0.027), presence of hypertensive emergencies at presentation (p=0.037), and evidence of chronic histologic changes i.e. sclerosis (p=0.007), interstitial fibrosis (p=0.003), tubular atrophy (p=0.027) and fibrous crescents (p=0.002), but not with the percentage of crescents per se (p=0.754).

Conclusions:

In context of RPGN, no laboratory investigation could neither be relied upon in prediction of severity of pathology, nor replace renal biopsy while taking a therapeutic decision. Additionally, the percentage of crescents beyond 20% did not prove to be a useful outcome indicator.