Abstract Details

Title An Alternative Regimen of Intravenous Immunoglobulin Monotherapy in Patients with Chronic Inflammatory Demyelinating Polyneuropathy and Dermatomyositis

Topic Peripheral Nerve

Presentation(s) P01 - (-)

Poster/Presentation
Number 149

Objective

Background BACKGROUND: Previous studies have confirmed a favorable response to IVIg in CIDP and DM. However, for treatment of CIDP, there are no defined recommendations on how best to taper the IVIg regimen and how long this treatment should continue once the patients improve. Additionally, the role of IVIg as a monotherapy in the treatment of DM is not well established. Through our retrospective study, we demonstrate an alternative treatment plan for CIDP and DM and address these issues.

Design/Methods DESIGN/METHODS: Retrospective review of 7 CIDP patients and 3 DM patients who were treated using weekly low doses of IVIg as a monotherapy (0.4 g/kg) started without loading doses and gradually tapered off treatment.

Results RESULTS: All (10 out of 10) patients showed signficant improvement in all categories from baseline to endpoint: modified Rankin score (CIDP -2.0, DM -1.3), MRC sum score (CIDP 12.8, DM 5.0), muscle dynamometer sum score (CIDP 94.3, DM 75.0), right hand grip strength (CIDP 30.0, DM 10.0), and left hand grip strength (CIDP 32.5, DM 3.0). All patients showed improvement within the first 24 weeks of treatment and 80% successfully reached a state of remission and are no longer receiving IVIg. The average length of treatment was 3.4 years for CIDP and 5.0 years for DM.

Conclusions CONCLUSIONS: We conclude that 1) a weekly low dose IVIg regimen without a loading dose is effective in treating both CIDP and DM and can be considered a first line and sole therapy for DM, 2) treatment can be individualized and tapered using the clinical exam as a guide, and 3) patients with CIDP and DM may reach disease-free remission with this dosing regimen, without the need for other immunosuppressive medications.

Authors/Disclosures
PRESENTER	No disclosure on file
Tahseen Mozaffar, MD, FAAN (University of California Irvine)	Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion Pharmaceuticals. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi-Genzyme. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Amicus. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Grifols. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Astellas Gene Therapy. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bayer. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Mozaffar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Applied Therapeutics. The institution of Dr. Mozaffar has received research support from NIH. The institution of Dr. Mozaffar has received research support from Muscular Dystrophy Association. The institution of Dr. Mozaffar has received research support from Sanofi. The institution of Dr. Mozaffar has received research support from Argenx. The institution of Dr. Mozaffar has received research support from Amicus Therapeutics. The institution of Dr. Mozaffar has received research support from Astellas Gene Therpay. The institution of Dr. Mozaffar has received research support from Cartesian. The institution of Dr. Mozaffar has received research support from Cabaletta. Dr. Mozaffar has received personal compensation in the range of $500-$4,999 for serving as a Study Section Member with NIH.
Gavin Giovannoni, MD (QMUL)	Dr. Giovannoni has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Giovannoni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi. Dr. Giovannoni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck KGaA. Dr. Giovannoni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche-Genentech. Dr. Giovannoni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Moderna. Dr. Giovannoni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sandoz. Dr. Giovannoni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Astoria Biologica. Dr. Giovannoni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Zenas. Dr. Giovannoni 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. Giovannoni has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Giovannoni has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Dr. Giovannoni has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Medscape.

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