متلازمة IPEX: إدارة المرحلة المناعية الذاتية الحادة عند عدم تحقيق الهدأة بعد زراعة الخلايا الجذعية المكونة للدم من متبرع خارجي

References

DOI: 10.3390/genes12030323

• Patients without HSCT are considered for immunosuppressive therapy (IS) and long-term supportive care.
• Immunosuppressive therapies are used with a combination of several immunosuppressive agents to control the acute autoimmune phase of IPEX syndrome.
• Patients after transplantation also receive immunosuppressive therapy, usually consisting of calcineurin inhibitors, cyclosporine A, sirolimus and tacrolimus, while some patients also receive steroids.
• The current study considered rapamycin, a non-calcineurin inhibitor, as the primary choice of immunosuppressive drug, either used alone or in combination.