The effectiveness of Omalizumab in the treatment of Hyper-IgE syndrome: A case report

Abstract

BACKGROUND - Hyper-immunoglobulin E (IgE) syndrome (HIES) is a rare primary multisystem immunodeficiency disorder with an incidence of one case per million population. It is characterized by elevated serum IgE levels, dermatitis, recurrent skin and lung infections, skeletal abnormalities such as scoliosis, and distinctive facial features. Moreover, the clinical manifestations may include vascular, dental, joint, and brain abnormalities. Treatment for HIES predominantly targets symptom relief. Omalizumab, a monoclonal antibody, works by inhibiting the binding of free IgE to receptors on effector cells, consequently reducing the release of inflammatory mediators and alleviating some cutaneous and respiratory symptoms of HIES. To date, according to our understanding, no treatment protocol for HIES patients exists in Jordan. To fill this gap, we report our experience using omalizumab and intravenous immunoglobulin (IVIG) in treating the first documented case of HIES in Jordan.  CASE PRESENTATION - We present the case of an 18-year-old male patient who had been diagnosed with HIES since infancy. The patient had been subjected to multiple treatment trials, but none proved effective. In our treatment protocol, 150 mg of omalizumab was administered subcutaneously every 2 weeks, later increasing to 300 mg. Following this treatment, the eczema symptoms and eosinophil count showed a marked reduction. The patient’s IgE level decreased from 21,800 IU/ml in 2011 and 2,039 IU/ml in 2019 to 764 IU/ml in 2022, with treatment commencing in the latter year.   CONCLUSION - The combined use of omalizumab and IVIG therapies demonstrated efficacy in reducing the elevated IgE levels and symptom severity in our patient. There is a need for more case studies to report clinical findings in the management of HIES