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Abstract Details

Use of Subcutaneous Immunoglobulin in Stiff Person Syndrome
Autoimmune Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)
15-003
SPS is a rare immune-mediated neurologic disorder. Multiple treatments are used for the treatment of SPS, although, to date, intravenous immunoglobulin (IVIG) remains the primary immunomodulating therapy in those who have inadequate responses to symptomatic therapies. However, in some patients, IVIG might not be tolerable or logistically difficult to administer. SCIg has emerged as an alternative to IVIG in certain disorders but to our knowledge, the use of SCIg has not been reported in SPS.
To describe our experience with subcutaneous immunoglobulin (SCIg) treatment in patients with stiff person syndrome (SPS).
This is a descriptive case series of patients with SPS who were treated with SCIg at Johns Hopkins. Review of medical records was from 1997 to 2019. Patients were included if they fulfilled clinical criteria for SPS and were exposed to SCIg. Patients were excluded if their symptoms were explained by another neurological disorder.
We identified five SPS patients that were treated with SCIg. Three patients tested positive for serum anti-GAD65 antibodies prior to any treatment. Mean age at SCIg initiation was 33 years (range:22-47). Mean duration of SPS prior to SCIg initiation was 5.9 years (range:2.5-7). All patients used IVIG for at least 2 months (up to 18 months) but switched to SCIg due to IVIG side effects. Duration of SCIg use ranged from 4 months to 6 years (mean,19.2 months). SCIg dose ranged from 0.4g-1g/kg/month. SCIg lead to sustained symptom improvement or stabilization in four patients (80%). The remaining patients had transient improvement. One patient developed mild-moderate injection site reactions.

This case series highlights that SCIg is a reasonable and safe immune-treatment option for SPS patients especially in whom IVIG is not feasible. Injection site reactions might be a limiting factor in some patients and future studies are needed to assess long-term safety/efficacy.

Authors/Disclosures
Salman Aljarallah, MD
PRESENTER
Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Sanofi Genzyme.
Scott Douglas Newsome, DO, FAAN (Johns Hopkins Hospital) Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Greenwich Biosciences. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. The institution of Dr. Newsome has received research support from Biogen. The institution of Dr. Newsome has received research support from Genentech/Roche. The institution of Dr. Newsome has received research support from Department of Defense. The institution of Dr. Newsome has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Newsome has received research support from National MS Society. The institution of Dr. Newsome has received research support from The Stiff Person Syndrome Research Foundation. The institution of Dr. Newsome has received research support from Lundbeck. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving as a Lead PI for Clinical Trial with Roche.