Romain Lévy, France
Institut Imagine INSERM UMR 1163Presenter of 1 Presentation
EFFICACY OF DUPILUMAB IN A PATIENT WITH SEVERE ALLERGY AND HYPER-IGE SYNDROME
Abstract
Background and Aims
Autosomal dominant form of the hyper-IgE syndrome is caused by STAT3 dominant-negative mutations, whereas autosomal recessive form is caused by loss-of-function mutations in ZNF341, which transcriptionally governs STAT3 activation. These patients present with severe allergy, including a severe form of eczema, which is thought to be caused by increased Th2 immunity. No effective treatment of their atopy is available besides topical steroids.
Methods
We treated a patient with ZNF341 deficiency and severe eczema with Dupilumab, blocking the alpha chain of the interleukin-4 receptor. Dupilumab was administered subcutaneously every 2 weeks for a year at 300 mg per dose. Every month, patient filled the DLQI and SCORAD standardized questionnaires to monitor the response. IgE levels and adverse events were monitored.
Results
At Dupilumab initiation, patient used steroids class I, DLQI was 22/30, and SCORAD was 85,5/100, defining a severe form of eczema. IgE were highly elevated at 16,689 kU/L. We observed a rapid improvement with a DLQI at 2, and a SCORAD at 9 after 2 months. Steroids were tapered and stopped after 3 months. After 1 year, the response was sustainable. Physical examination noted a remarkable improvement. Patient no longer suffered from scratching lesions. Nodular lesions disappeared, and epithelialization occurred in depigmented areas. No adverse effects were noted. IgE progressively decreased, up to 6022 kU/L.
Conclusions
Dupilumab is a safe and efficient therapy in patients with genetically driven severe eczema. Moreover, this observation furnishes a rational for the use of Dupilumab in other patients with inborn error of immunity and Th2-mediated severe allergy.