Nephrotic syndrome in childhood and adolescence. Summary of the AWMF guidelines considering special aspects of diagnostics and treatment in adults

Background. Despite fulfilling the criteria for an orphan disease, idiopathic nephrotic syndrome (NS) in children is the most frequent glomerular disease in this age group. At the same time NS in children is aheterogeneous disease and in order to assess the individual facets of the disease aclassification using the following criteria is helpful: etiology, age at onset, histology and responsiveness to initial standard treatment with glucocorticoids. As for prognosis the differentiation between steroid-sensitive (SSNS) and steroid-resistant nephrotic syndromes (SRNS) is most important because SRNS is arisk factor for developing end-stage renal disease. High cumulative doses of glucocorticoids or prolonged administration in the initial treatment do not influence the risk of a relapse. Genetic testing is highly advisable in patients with SRNS to avoid unnecessary and ineffective immunosuppressive therapy. Methods. This manuscript gives an overview on the management of SSNS and SRNS and provides insights into the different diagnostic and therapeutic approaches in pediatric and adult medicine. Furthermore, associated risks and potential long-term side effects of treatment are described. Conclusion. Frequently relapsing NS and SRNS are still challenging to treat. The use of steroids and immunosuppressive maintenance therapy has severe long-term side effects. The optimal treatment of adult patients with childhood onset SSNS and infrequent relapses remains unclear.