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Objective.  To assess whether high-molecular-weight proteins excretion predicts outcome and therapy-responsiveness in patients with FSGS and nephrotic syndrome.  Research Design and Methods . Thirty-eight patients measured at biopsy fractional excretion of IgG (FEIgG) and urinary   α  2-macroglobulin/creatinine ratio (  α  2m/C). Low and high risk groups were defined by cutoffs assessed by ROC analysis. In all patients first-line therapy was with steroids alone or in combination with cyclophosphamide.  Results.α  2m/C and FEIgG were correlated with segmental sclerosis ( r  = 0.546;  r  = 0.522). Twenty-three patients (61%) entered Remission and 9 (24%) progressed to ESRD. Comparing low and high risk groups, by univariate analysis remission was predicted by FEIgG (77% versus 25%,  P  = 0.016) and   α  2m/C (81% versus 17%,  P  = 0.007) and ESRD at best by FEIgG (0% versus 75%,  P  < 0.0001) and   α  2m/C (4% versus 67%,  P  < 0.0001). By multivariate analysis FEIgG was the only independent predictor of remission and   α  2m/C the most powerful predictor of ESRD. Low and high risk groups of FEIgG and   α  2m/C in combination had very high predictive value of sustained remission and ESRD in response to therapy.  Conclusions.  FEIgG and   α  2m/C are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide; their predictive value, if validated in prospective studies, may be useful in clinical practice suggesting first-line alternative treatments in high risk patients.

biomed research international

Urinary IgG and<b><i>α</i></b>2-Macroglobulin Are Powerful Predictors of Outcome and Responsiveness to Steroids and Cyclophosphamide in Idiopathic Focal Segmental Glomerulosclerosis with Nephrotic Syndrome

Urinary IgG andα2-Macroglobulin Are Powerful Predictors of Outcome and Responsiveness to Steroids and Cyclophosphamide in Idiopathic Focal Segmental Glomerulosclerosis with Nephrotic Syndrome