Abstract
Immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder caused by mutations in the FOXP3 gene that result in the defective development of CD4+CD25+ regulatory T cells. In the absence of CD4+CD25+ regulatory T cells, activated CD4+ T cells instigate multi-organ damage. IPEX syndrome is often initially treated with immunosuppressive drugs, but only allogeneic hematopoietic stem cell transplantation has offered the possibility of cure. Kidney complications in IPEX syndrome patients have been reported to be membranous nephropathy, tubulointerstitial damage and minimal change nephritic syndrome (MCNS). We suspected that this complication is caused by a disorder of a T cell function due to IPEX syndrome. Now we report the relationship between IPEX syndrome and its complication of the kidney disease from a point of view of regulatory T cells.
