Retrospective Review of the Utilization and Clinical Monitoring of Immunoglobulin Therapy in Patients with Stiff-person Syndrome in a Home Infusion Setting
Timothy Walton1, Elizabeth Duruz1, Carol Sauerbrun1, Elizabeth Neal1, James Sheets1, Randy Broyles1, Leslie Myers1, Michele Way1, Edward O'Bryan1
1CSI Pharmacy
Objective:
To evaluate Immunoglobulin (Ig) therapy monitoring and clinical outcomes in Stiff-Person Syndrome (SPS) patients receiving home infusion therapy.
Background:
SPS is a rare neurological disorder (1–2 cases per million) with a suspected autoimmune basis. Symptoms include rigidity, pain, and spasms triggered by sensory or emotional stimuli. IVIg is the primary treatment, with SCIg as an alternative. Home infusion providers support access, payor approvals, and clinical oversight, yet limited data exist on SPS management in non-hospital settings.
Design/Methods:
A retrospective review was conducted in SPS patients treated from 1/2016-5/2025. Data from the proprietary clinical outcomes program, Clinical Outcomes 360°® assessed demographics, dosing patterns, clinical outcomes, and Adverse Drug Reactions (ADRs) collected through pharmacist-led interviews and nursing documentation. Descriptive analyses summarized findings.
Results:
Seventy-three SPS patients received home infusion Ig therapy 94% IVIg and 6% SCIg. The cohort was 83.6% female, with median age 58. Median IVIg dosing was 1.3 g/kg/ every 4 weeks, SCIg 0.99g/kg/month (0.25g/kg weekly) over 6 injection sites, with 1271 dispenses filled (1210 IVIg/61 SCIg). Common symptoms throughout treatment included stiffness (82%), muscle weakness (79%), fatigue (77%), poor balance (65%), unsteady gait (64%), and muscle spasms (40%). Falls occurred in 3% of assessments and median pain score was 3 on a 1-10 scale. ADRs were mostly mild or moderate, including headache, fatigue, fever, hypertension, anemia, nausea, and Infusion Site Reactions (ISRs) of swelling, itching, rash, and redness. Serious ADRs were rare (headache and systemic hypersensitivity reactions) and no critical ADRs were reported.
Conclusions:
Home infusion supports SPS care, with IVIg preferred and SCIg a potential alternative. Symptoms impact mobility and quality of life. Most ADRs and ISRs were mild; serious events were rare. No critical ADRs occurred. Structured monitoring is essential. Future research should compare Ig therapies, develop standardized Patient Reported Outcome Measures (PROMs), and improve access and long-term outcome tracking.
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