Successful use of the new immune-suppressor sirolimus in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome)
L Bindl, T Torgerson, L Perroni, N Youssef… - The Journal of …, 2005 - Elsevier
L Bindl, T Torgerson, L Perroni, N Youssef, HD Ochs, O Goulet, FM Ruemmele
The Journal of pediatrics, 2005•ElsevierIPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is an
autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy.
We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is
probably due to its different mode of action compared to calcineurin-dependent agents.
autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy.
We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is
probably due to its different mode of action compared to calcineurin-dependent agents.
IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is an autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy. We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is probably due to its different mode of action compared to calcineurin-dependent agents.
Elsevier
