FOXP3

Management

The only curative treatment currently available for IPEX is hematopoietic stem cell transplantation. Individuals who do not undergo transplantation are treated with immunosuppressive therapy, as well as supportive therapies for autoimmune symptoms, dermatitis or enteropathy/diarrhea. For prevention of secondary complications, prophylactic antibiotic therapy or aggressive management of dermatitis is recommended, depending on clinical symptoms. For surveillance, periodic evaluation of complete blood counts, glucose tolerance, thyroid function, kidney and liver function to monitor for autoimmune disease.