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Abstract Details

Investigating the Role of Sjogren’s Syndrome-associated Antibodies in Inclusion Body Myositis
Neuromuscular and Clinical Neurophysiology (EMG)
P3 - Poster Session 3 (11:45 AM-12:45 PM)
11-014

1) Determine the incidence of positive anti-SSA (Ro) and/or anti-SSB (La) antibodies in our population of patients with inclusion body myositis, and 2) correlate anti-SSA and/or anti-SSB antibody positivity with clinical and serologic features.

Inclusion body myositis (IBM) is a slowly progressive muscle disorder that causes significant morbidity and does not have any effective treatment.  There is substantial evidence for a primary inflammatory contribution to the disease pathogenesis, with circulating and muscle immune cell populations that are skewed towards highly differentiated T-lymphocytes, cytotoxic auto-invasive lymphocytic and plasma/dendritic cell infiltrates in the muscle, HLA-DR3 haplotype association and circulating NT5c1A autoantibodies that have high specificity.  IBM also shares an association with other inflammatory and autoimmune diseases, such as Sjögren’s disease.  Prior epidemiological studies have estimated that 6-10% of patients with IBM have evidence of Sjogren’s syndrome. There are case reports of patients with Sjogren’s syndrome-associated IBM that have demonstrated improved strength after immunosuppression.  Some of these cases are treatment responsive initially to conventional immunosuppressive therapy but then lose response, as autonomous muscle degeneration begins to occur.

INSPIRE-IBM is the largest and longest natural history study in IBM, funded through NIAMS/NIH and has enrolled 150 subjects across 13 sites in the US. Our institution is one of these sites and we have a large number of patients with IBM that follow-up in our multidisciplinary MDA clinic.  We will retrospectively analyze the rates of anti-SSA and anti-SSB antibody positivity in our population of patients with IBM.  We will also investigate associations between anti-SSA/anti-SSB antibody positivity, NT5c1A antibody positivity and clinically relevant data such as the IBMFRS or MMT12 scores.

Data collection ongoing.
To be determined.
Authors/Disclosures
Samuel Frank, MD (Providence Mission Heritage Medical Group)
PRESENTER
Dr. Frank has nothing to disclose.
Namita Goyal, MD, FAAN (University of California, Irvine) Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Annexon. Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abcuro. Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Souffle. Dr. Goyal has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for CSL Behring. Dr. Goyal has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx. Dr. Goyal has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Amgen. The institution of Dr. Goyal has received research support from Fulcrum. The institution of Dr. Goyal has received research support from Healey Platform. The institution of Dr. Goyal has received research support from Medicinova. The institution of Dr. Goyal has received research support from Transposon. The institution of Dr. Goyal has received research support from Abcuro. The institution of Dr. Goyal has received research support from Calico. The institution of Dr. Goyal has received research support from Clene. The institution of Dr. Goyal has received research support from Janssen. The institution of Dr. Goyal has received research support from PepGen. The institution of Dr. Goyal has received research support from PTC. The institution of Dr. Goyal has received research support from Roche. The institution of Dr. Goyal has received research support from Sanofi. The institution of Dr. Goyal has received research support from Prilenia. The institution of Dr. Goyal has received research support from RAPA. The institution of Dr. Goyal has received research support from Avidity. Dr. Goyal has received publishing royalties from a publication relating to health care.
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.