BACKGROUND: We report a 7-year-old boy with high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) who went into remission with rituximab (RTX) maintenance therapy. CASE-DIAGNOSIS/TREATMENT: Four months after this patient received his first RTX infusion, there was a progressive and sustained decrease of immunoglobulin (Ig)G and IgM levels. Thirteen months after the initiation of RTX therapy he was in sustained remission without any steroid or oral immunosuppressive therapy; however, B cell depletion was still present. At this time he developed a fulminant myocarditis due to enterovirus. Despite aggressive treatment and the administration of intravenous polyvalent immunoglobulins there was no clinical improvement. He successfully underwent heart transplant surgery. CONCLUSIONS: We conclude that B cell depletion with RTX is efficacious in the treatment of paediatric SDNS but that it may be associated with severe infectious complications. Therefore, we recommend a close monitoring of Ig levels in children who have received RTX therapy and a supplementation with intravenous Ig as soon as the Ig levels fall below the lower limit of the normal range.
BACKGROUND: We report a 7-year-old boy with high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) who went into remission with rituximab (RTX) maintenance therapy. CASE-DIAGNOSIS/TREATMENT: Four months after this patient received his first RTX infusion, there was a progressive and sustained decrease of immunoglobulin (Ig)G and IgM levels. Thirteen months after the initiation of RTX therapy he was in sustained remission without any steroid or oral immunosuppressive therapy; however, B cell depletion was still present. At this time he developed a fulminant myocarditis due to enterovirus. Despite aggressive treatment and the administration of intravenous polyvalent immunoglobulins there was no clinical improvement. He successfully underwent heart transplant surgery. CONCLUSIONS: We conclude that B cell depletion with RTX is efficacious in the treatment of paediatric SDNS but that it may be associated with severe infectious complications. Therefore, we recommend a close monitoring of Ig levels in children who have received RTX therapy and a supplementation with intravenous Ig as soon as the Ig levels fall below the lower limit of the normal range.
Authors: Markus J Kemper; Jutta Gellermann; Sandra Habbig; Rafael T Krmar; Katalin Dittrich; Therese Jungraithmayr; Lars Pape; Ludwig Patzer; Heiko Billing; Lutz Weber; Martin Pohl; Katrin Rosenthal; Anne Rosahl; Dirk E Mueller-Wiefel; Jörg Dötsch Journal: Nephrol Dial Transplant Date: 2011-11-09 Impact factor: 5.992
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Authors: Nils Venhoff; Nora M Effelsberg; Ulrich Salzer; Klaus Warnatz; Hans Hartmut Peter; Dirk Lebrecht; Michael Schlesier; Reinhard E Voll; Jens Thiel Journal: PLoS One Date: 2012-05-21 Impact factor: 3.240