MULTICENTER STUDY OF OUTCOME INDICATORS IN CHILDREN WITH SEVERE CRESCENTIC GLOMERULONEPHRITIS (CrGN)

Abstract

Previous studies from small series of patients have suggested that the prognosis of children with CrGN may be predicted on the basis of % glomeruli (G) with crescent (Cr) formation, rather than the underlying type of GN. We have re-evaluated this issue in 50 children with a variety of renal diseases in whom renal biopsies showed Cr in ≥50% of G. 67 clinical and 53 biopsy variables were analyzed in 30 girls and 20 boys with a mean age of 10.1 yrs (range 1.7-17.2 yrs). Presenting features included edema in 61%; hypertension (HT) in 51%; gross hematuria in 73%; 3-4+ proteinuria in 78%; and ↓↓GFR (<30 ml/min/ 1.73m2) in 66%. When the total group was divided into those with 50-79% Cr (n=18) and those with 80-100% Cr (n=32), no difference in outcome could be demonstrated, with end-stage renal disease (ESRD) being seen in 44% and 50% of the two gps. Features that did indicate a poor prognosis included G sclerosis (p=0.05); G IgM (p=0.003); interstitial fibrosis (p=0.03); tubular atrophy (p=0.04); predominance of large Cr (p=0.004) or fibrous Cr (p=0.03) and ↑frequency of gaps in Bowman's capsule (p=0.004). Persistent ↓↓GFR was not seen in 6/6 pts with post-strep GN, but present in 60% of pts with other conditions. We conclude (1) pts with severe Cr GN often progress to ESRD and (2) helpful prognostic indicators include the underlying type of GN and evidence of chronic histologic changes, but not the percent of glomerular crescents.