Nephrotic syndrome refractory to conventional therapy

BACKGROUND: Prednisone is the choice medicine in Nephrotic Syndrome (NS) treatment, possibly associated with immunosuppressor medicines (cyclophosphamide or chlorambucil), either in case of NS resistance at cortisone therapy or with frequent relapses. Cyclosporin A (CyA) use has been recently proposed, due to its inhibitory effect on the IL2 and lymphokine release, with a permeabilizing effect on the glomerular membrane. The purpose of this study is to evaluate the CyA antiproteinuric effectiveness with NS conventional therapy refractory patients. METHODS: Six patients (3 females and 3 males) have been treated with CyA (4 +/- 0.5 mg/Kg/die) associated with low corticosteroid dosages. RESULTS: During the treatment, proteinuria reduced in 5 patients, at less than 1/3 of pre-treatment values, for 4 patients this happened starting from the 2nd month of therapy, while after the 12th for the fifth patient. The sixth patient has now a 2/3 reduction compared to the initial one and he is at the 3rd month of therapy. During the CyA treatment, further to the proteinuria reduction, a total proteinemia values increase and a cholesterolemia and tryglyceridemia reduction has been observed, while creatinine and PA have not changed. CONCLUSIONS: Four out of the six treated patients have been respectively under therapy for 2,3,12,30 months. Two stopped CyA therapy: one after 18 months due to clinical stability, still present after 2 years from interruption; one after 9 months with a stable clinical picture for just three months, since she was longing for a pregnancy, achieving a quick proteinuria relapse.