Lupus nephritis in children: Prognostic significance of clinicopathological findings

Background: We aimed to review our experience with childhood lupus nephriti s (LN) in respect to the analysis of the clinical and histopathological pre sentation of LN and prognostic factors affecting the kidney and patient out comes. Method: Forty-three children (39 girls, 4 boys) with biopsy-proven L N were included in the study. The mean age of the children was 12.0 +/- 2.8 years. Based on the renal histopathology and clinical presentation, patien ts were treated with oral prednisone, intravenous pulses of methylprednisol one or intravenous cyclophosphamide. The final clinical status was classifi ed as follows: (1) renal and extrarenal remission; (2) clinically active re nal disease, or (3) adverse outcome, i.e., endstage renal failure (ESRF) or death. Results: The mean duration of follow-up was 7.2 +/- 2.8 years (1 mo nth to 14.2 years). All 43 children had hematuria and 53.5% had proteinuria at admission. Fourteen children were in nephrotic status at the onset of d isease. Class IV (diffuse proliferative) nephritis was observed in 29 patie nts as the most frequent histopathology (67.4%). The patients with class IV nephritis had a tendency to develop nephrotic syndrome, heavy proteinuria, increased Cr levels and persistent hypertension at initial evaluation. Thi rty-two of 43 children (74.4%) were in renal remission at the last visit. F ive-year kidney and patient survival rates from the time of diagnosis to th e endpoints of ESRF or death were 83.7 and 90.7% respectively in the whole group while it was 75.9 and 86.2% respectively in the class IV group. Adver se outcome was significantly associated with the persistent hypertension, a nemia, high serum Cr level, heavy proteinuria, nephrotic syndrome and class IV nephritis at presentation. Conclusion: We can conclude that the prognos is of LN in children is primarily dependent on the histopathological lesion s. Severity of the clinical renal disease at admission and presence of pers istent hypertension are the main poor prognostic factors rather than age, g ender, low C3 and C4 levels, ANA positivity and the treatment modalities in Turkish children. Copyright (C) 2001 S. Karger AG, Basel.