The Immune System and Idiopathic Nephrotic Syndrome

Idiopathic nephrotic syndrome often responds to immunosuppressive treatment. Nevertheless, this syndrome?and the drugs used to treat it?remain important causes of patient morbidity. Idiopathic nephrotic syndrome is usually caused by minimal change disease or FSGS, diseases that primarily affect the podocytes. In spite of decades of research, the underlying causes of both diseases remain incompletely understood. There is, however, a large body of observational and experimental data linking the immune system with both minimal change disease and FSGS, including associations with systemic infections and hematologic malignancies. Perhaps most compellingly, many different immunomodulatory drugs are effective for treating idiopathic nephrotic syndrome, including biologic agents that have well-defined immune targets. In fact, the unexpected efficacy of targeted therapeutic agents has provided important new insights into the pathogenesis of these diseases. Given the large number of drugs that are available to deplete or block specific cells and molecules within the immune system, a better understanding of the immunologic causes of idiopathic nephrotic syndrome may lead to better diagnostic and therapeutic approaches.

Automated summary

Idiopathic nephrotic syndrome often responds to immunosuppressive treatment, but remains a significant cause of patient morbidity. Minimal change disease and FSGS are the usual causes of the syndrome, primarily affecting podocytes. Although the underlying causes of both diseases are not completely understood, there is evidence linking the immune system to both diseases. Immunomodulatory drugs, including biologic agents, have shown efficacy in treating idiopathic nephrotic syndrome and have provided insights into the diseases' pathogenesis.