Rituximab can induce remission of nephrotic syndrome in the absence of peripheral B‐cells

remission. In NF1, the mechanism of glomerulopathy is unknown. However, inflammatory signaling pathways such as MAPK and mTOR secondary to upregulation of ras proteins due to deficient neurofibromin might be implicated in the pathogenesis of this condition. In conclusion, this is the first case report of nephrotic syndrome with IgM nephropathy in a patient with NF1, and our patient was successfully treated with steroids. Conflicts of interest: We hereby declare that we do not have any conflict of interest.

[1]  M. Gos,et al.  Neurofibromin in neurofibromatosis type 1 - mutations in NF1gene as a cause of disease. , 2014, Developmental period medicine.

[2]  J. Deegens,et al.  B cell suppression in primary glomerular disease. , 2014, Advances in chronic kidney disease.

[3]  H. Koenen,et al.  A Single Dose of Rituximab Does Not Deplete B Cells in Secondary Lymphoid Organs but Alters Phenotype and Function , 2013, American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons.

[4]  T. Mochizuki,et al.  Effect of single-dose rituximab on steroid-dependent minimal-change nephrotic syndrome in adults. , 2013, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[5]  V. Vega-Warner,et al.  Focal segmental glomerulosclerosis in association with neurofibromatosis type 1: a case report and proposed molecular pathways , 2013, Clinical kidney journal.

[6]  Mei-Mei Cheng,et al.  Minimal Change Disease in a Patient with Neurofibromatosis Type I , 2012 .

[7]  M. Rastaldi,et al.  Rituximab Targets Podocytes in Recurrent Focal Segmental Glomerulosclerosis , 2011, Science Translational Medicine.

[8]  Steven Z. Athanases,et al.  Clinical and genetic aspects of neurofibromatosis 1 , 2010, Genetics in Medicine.

[9]  F. McCormick,et al.  Ras regulation. NF is enough of GAP. , 1992, Nature.