Clinica Pediatrica, Policlinico Universitario di Udine
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Key words: Steroid-sensitive nephrotic syndrome, Prednisone, Levamisole, Cyclophosphamide, Cyclosporin, Mycophenolate mofetil
The steroid treatment of Idiopathic Nephrotic Syndrome (ISN) should be continued for at least 3 months, as there is evidence of an inverse correlation between the duration and the total dosage of steroids and the risk of relapses. The tendency to relapses is a characteristic of the natural history of ISN and only less than 10% of patients experience one attack. In contrast with what happens in the treatment of the first episode, the intensity of the therapy during relapses does not influence the recurrence rate. There are two initial therapeutic options for frequent relapsing and steroid-dependent ISN: 1) repeated treatments according to the standard therapeutic regime for relapses or 2) the “discontinued prolonged corticosteroid therapy”. Well-tolerated steroid therapy can be maintained for as much as 10-15 years, without the necessity of other drugs. Alternative therapy should be considered in the following cases: severe adverse effects of prednisone, treatment during puberty, when the risk of steroid toxicity is higher, major complications during severe relapses, inadequate follow-up and poor compliance. Drugs effective in reducing the relapse rate, as demonstrated in randomised controlled trials, are cyclophosphamide (CYC), chlorambucil (CHL), cyclosporin (CyA) and levamisole. The latter can be considered the first choice alternative to steroids as it is well tolerated and rarely induces adverse effects. Most authors recommend not introducing CyA before trying CYC, as there is the possibility of definite and long-term remission with CYC. Mycophenolate mofetil (MMF) is a promising drug, although more controlled and multicentric trials to confirm its advantages on CyA, especially in terms of adverse effects are needed.
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