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IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndr ome is an autoimmune disorder with an often lethal outcome in spite of immunosup pressive therapy. We report the successful use of sirolimus in 3 patients with I PEX. The efficacy of sirolimus is probably due to its different mode of action c ompared to calcineurin-dependent agents. Copyright
IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndr ome is an autoimmune disorder with an often lethal outcome in spite of immunosupressive therapy. We report the successful use of sirolimus in 3 patients with I PEX. The efficacy of sirolimus is probably due due to its different mode of action c ompared to calcineurin-dependent agents. Copyright