Article ID Journal Published Year Pages File Type
5664535 Transfusion and Apheresis Science 2017 6 Pages PDF
Abstract

About 30% of the cases of steroid resistant nephrotic syndrome display a genetically determined disease and will not recur after kidney transplant; the other cases with fully or partially immunological pathogenesis display a high risk of post transplant recurrence.Although lots of studies were carried out in the last 50 years the pathogenetic mechanism is still obscure and the therapeutic approach mostly empirical. The cornerstones principles of the therapies are based on removal of a still undefined “permeability factor” through plasma-exchange or other apheresis techniques and inhibition of its synthesis by the immunological system through different drugs.The probability of successfully inducing persistant remission is nowadays around 30%through the different schemes experimented so far which mostly include plasmapheresis. Rituximab in the last years has significantly increased the efficacy of the treatments.Non responders are rapidly evolving to graft loss and will most probably recur also in subsequent transplant.Apart from genetics no other risk factors are predictive for recurrence.

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