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Remission of proteinuria in multidrug‐resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption
Author(s) -
Nattes Elodie,
Karaa Danièle,
Dehoux Laurène,
Peuchmaur Michel,
Kwon Theresa,
Deschênes Georges
Publication year - 2019
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/apa.14582
Subject(s) - medicine , nephrotic syndrome , immunoadsorption , prednisone , rituximab , proteinuria , immunosuppression , gastroenterology , antibody , immunology , kidney
Aim Complete or partial resistance to prednisone and calcineurin inhibitors in children with idiopathic nephrotic syndrome often leads to end‐stage renal disease. The aim of the study was to report the outcome of patients with multidrug‐resistant nephrotic syndrome treated with an association of immunoglobulin immunoadsorption, intravenous immunoglobulins and B‐cell depletion. Methods At treatment initiation, patients received ten sessions of immunoglobulin immunoadsorption and intravenous immunoglobulins in two weeks followed by one rituximab in case of remission. Results A remission of proteinuria was obtained in nine out of 14 patients at the end of the initial phase and in two additional patients after an extended period. The remission was stable in three patients and after additional Ig IA and heavy immunosuppression in six. Two patients that initially responded relapsed after Ig IA withdrawal and remained with an uncontrolled disease at last follow‐up. Three patients did not respond to the treatment including two who were found to have a genetic podocytopathy. Conclusion Patients with multidrug‐resistant idiopathic nephrotic syndrome can be successfully led into remission by Ig IA prior to reaching end‐stage renal disease. However, Ig IA does not suppress the need for heavy additional immunosuppression to control INS in most cases.