Cyclosporin A in hyperimmunoglobulin E syndrome

Background: The hyperimmunoglobulinemia E syndrome is a serious disorder of an unknown etiology without effective treatment. Cyclosporin A has immunosuppressive properties. Its effect in a patient with hyperimmunoglobulinemia E syndrome was evaluated. Methods: We report a 3- year-old boy with severe hyperimmunoglobulinemia E syndrome in whom various therapeutic modalities were ineffective. He was started on cyclosporin A 3 mg/kg/d for 6 months. We examined several humoral and cellular immune functions during the treatment period and monitored his clinical condition. Results: Marked improvement in his clinical condition was observed during therapy with cyclosporin A. There was a significant decrease in serum IgE and significant increase in neutrophil chemotactic function. No change in CD40 ligand expression was observed. Cyclosporin A blood level did not exceed 150 ng/mL during the study. Conclusions: A small dose of cyclosporin A (3 to 5 mg/kg/d) is beneficial in patients with hyperimmunoglobulinemia E syndrome. It should be considered in severe cases where other therapeutic modalities have failed.