Long-term renal outcome in pediatric glomerulonephritis associated with crescent formation

Background

Information on long-term renal outcome of pediatric glomerulonephritis associated with crescent formation is limited. A single center retrospective study was conducted to assess long-term renal survival and to determine whether the 2010 classification for antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis can predict renal outcome in pediatric glomerulonephritis associated with crescent formation.

Methods

Biopsy and clinical data of children, aged ≤ 18 years with ≥ 10 glomeruli and ≥ 10% crescentic glomeruli during January 1998 to December 2015, were reviewed. Biopsies were classified according to the 2010 classification into focal, crescentic, mixed, and sclerotic classes. The clinical endpoint was end-stage renal disease (ESRD).

Results

Of 72 children, 14 patients (19.4%) had positive ANCA. The biopsy indication was rapidly progressive glomerulonephritis in 38 patients (52.8%) and 22 patients (30.6%) required dialysis at onset. Lupus nephritis was the most common diagnosis (43.1%), followed by IgA nephropathy/Henoch–Schoenlein purpura (HSP) (22.2%). ESRD occurred in 18 patients (25%) and the risk of ESRD differed among the histological classifications (p < 0.001). Dialysis at onset and sclerotic class was independent predictors of ESRD in an adjusted model. The risk of ESRD was four-fold higher in patients requiring dialysis at onset and 7.7-fold higher in sclerotic patients than in crescentic patients.

Conclusions

The probability of ESRD was substantial in pediatric glomerulonephritis associated with crescent formation. The 2010 classification is useful for establishing long-term renal prognosis. Future research is required to validate whether histological classification could be a determinant in therapeutic guideline modification, since long-term renal prognosis is different in each class.