HDCN Article Review/Hyperlink

Baqi N, Moazami S, Singh A, Ahmad H, Balachandra S, Tejani A

Lupus nephritis in children: A longitudinal study of prognostic factors and therapy

J Am Soc Nephrol (Jun) 7:924-929 1996

Baqi et al performed a retrospective study of 56 children with lupus nephritis at SUNY-Brooklyn under the age of 18 (34% less than 12 years). All patients underwent renal biopsy, graded according to WHO classification. From 1965-1985, Class III and IV lesions were treated with high dose or pulse steroids; from 1985-1992, intravenous cyclophosphamide was also given for these lesions.

Fifty percent of all patients developed ESRD with a mean follow-up of 5.9 years. They were compared to patients who did not progress, followed up for a mean of 4.7 years. ESRD was significantly more likely to occur in children with highest initial serum creatinine level, presence of hypertension at diagnosis, and Class IV histology. Median renal survival time for Class IV histology was 2.9 years compared to 7.2 years for those with Class I-III, however, 42% of their patients presented already with Class IV disease. Using multivariate stepwise logistic regression analysis, Class IV biopsy presentation was the most predictive indicator for the development of ESRD, followed by hypertension. The combination of low C-3 complement level with elevated serum creatinine was a significant independent predictor of ESRD.

The authors point out that while several studies suggest decreasing mortality in children with lupus nephritis, they confirm their earlier observation that their patient population, with more African-American and younger patients, had higher rates of morbidity and mortality. One important conclusion of this study supports the observation that African-American children with lupus nephritis have more severe disease with a "dismal" prognosis. This study improved on previous reports by using only new cases and adding renal biopsy data into the clinical model. Importantly, the addition of intravenous cyclophosphamide conferred no additional benefit in preventing progression to end stage renal disease in Class III and IV patients. This may have been related to the predominance of African-American children. The importance of identifying and aggressively treating hypertension at the onset of lupus and the importance of renal biopsy in providing prognosis, particularly in African-American children with Class IV disease were stressed.

Comment: While there will be little argument that aggressive management of hypertension is essential, this study raises important questions about the role of cyclophosphamide in the treatment of lupus nephritis in children. As noted by the authors, a randomized controlled, multi-center study is necessary to determine the most effective treatment, particularly of Class IV disease in children. (N. Kevin Krane, M.D., Tulane University)