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Abstract Details

Anti-cN1A Antibodies do not Help Characterize sIBM
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
111
To analyze clinical, serological, electrophysiological and myopathological differences of patients with sporadic inclusion body myositis (sIBM) in relation to presence or absence of anti-cytosolic 5’-nucleotidase 1A (cN1A) antibodies and their titer.
Anti-cN1A antibodies are commonly detected in patients with sIBM. However, their pathogenic role has not been established. Moreover, efforts towards identifying sIBM distinct clinicopathologic characteristics associated with these antibodies have yielded conflicting results.
We first searched our institute database to look for patients tested for anti-cN1A antibodies, and evaluated in our clinics between December 2015 and December 2019, to identify sIBM patients. Thereafter, we reviewed and compared the clinical and investigational findings of these patients in relation to their antibody status. We excluded sIBM patients also seen in our clinics but not tested for anti-cN1A antibodies.
We identified 92 patients who were diagnosed with sIBM, according to the 2011 ENMC criteria or Griggs et al criteria. Anti-cN1A antibodies were present in 47/92 (51%) patients with sIBM. Comparison of seropositive and seronegative cohorts yielded no statistically significant difference in disease severity or clinical features, including facial weakness, oropharyngeal or respiratory involvement. The antibody titer did not correlate with the creatine kinase value or presence of myotonic discharges. Anti-cN1A antibody positive patients had more frequent auto-aggressive inflammation (100% vs 87%, p =0.01) on muscle biopsy.
Our study showed that anti-cN1A antibody positive and negative sIBM patients have similar clinical, serological and electrophysiological features and disease severity, including degree of oropharyngeal dysphagia. Although, on biopsy,  seropositive sIBM patients were more likely to have an auto-aggressive inflammatory exudate,  this did not occur as  an isolated myopathological feature. Therefore, anti-cN1A antibodies in our sIBM cohort were of limited value in patients’ assessment.
Authors/Disclosures
Pritikanta Paul, MD (University of California, San Francisco)
PRESENTER 		The institution of Dr. Paul has received research support from ZS Associates.
Teerin Liewluck, MD, FÂ鶹´«Ã½Ó³»­ (Department of Neurology, Mayo Clinic) Dr. Liewluck has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta Therapeutics. Dr. Liewluck has received publishing royalties from a publication relating to health care.
No disclosure on file
Jayawant N. Mandrekar, PhD Dr. Mandrekar has nothing to disclose.
Margherita Milone, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Milone has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cartesian Therapeutics. Dr. Milone has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology Genetics, Â鶹´«Ã½Ó³»­. The institution of Dr. Milone has received research support from Mayo Clinic, CCaTS-CBD. The institution of Dr. Milone has received research support from Mayo Clinic, SGP Award. The institution of Dr. Milone has received research support from MDA for Care Center grant. The institution of Dr. Milone has received research support from Regenerative medicine Minnesota.