Abstract

Objective: To report the use of 20% proline-stabilized subcutaneous immunoglobulin (SCIG; Hizentra®) in 2 patients with myasthenia gravis (MG) and 1 patient with chronic inflammatory demyelinating polyneuropathy (CIDP) for whom intravenous immunoglobulin (IVIG) was poorly tolerated or ineffective. Background Guidelines suggest the use of IVIG for treatment of MG and CIDP. However, some patients experience intolerable adverse events or lack of efficacy with IVIG administration. SCIG therapy is used and tolerated in immunodeficient patients. The high volume used in neurologic disease has previously limited SCIG use. Hizentra is the highest concentration SCIG available (20% IgG), allowing for reduced volumes. Design/Methods: Patient 1 is a 14 year-old boy with CIDP who experienced severe headaches and vomiting for up to 8 days after IVIG treatment (1 g/kg/mo) interfering with daily activities. Patient 2 is a 35 year-old female with MG who experienced intolerable headaches with maintenance IVIG (1 g/kg/mo), resulting in poor compliance. Patient 3 is a 13 year-old girl with MG whose symptoms were not improving after IVIG (0.5 g/kg/mo). Results: Patient 1 is treated with 250 mg/kg/wk Hizentra (25 ml/site/4 sites, 2X/wk) and reports improved strength and ability to participate in daily activities, with no adverse effects. Patient 2 is treated with 250 mg/kg/wk Hizentra (25 ml/site/2 sites, 2X/wk) and reports improved energy and MG symptoms, with no adverse effects. Patient 3 is treated with 125 mg/kg/wk (20 ml/site/3 sites, 1X/wk) and reports improved energy, with no adverse effects. Conclusions: SCIG was well tolerated and improved symptoms and quality of life in neurology patients intolerant or unresponsive to IVIG. More research is needed to further assess efficacy in chronic neurological diseases. SCIG should be considered as an alternative to IVIG in certain patients. Disclosure: Ms. Zampelli has received personal compensation for activities with CSL Behring. Dr. Zacek has received personal compensation for activities with CSL Behring. Ms. Levasseur has received personal compensation for activities with CSL Behring as a consultant.

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