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Abstract Details

A 40-year population-based epidemiologic study of sporadic inclusion body myositis (sIBM) in Olmsted County, Minnesota
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
113

To investigate the prevalence of sporadic inclusion body myositis (sIBM), outcomes, and overall survival. 

The prevalence of sIBM varies from country to country, ranging from 0.1 to 13.93/100,000 people ≥ 50 years old. Recent data on the prevalence of sIBM in the US is lacking.

We identified sIBM patients, fulfilling the 2011 European Neuromuscular Centre (ENMC) diagnostic criteria, among Olmsted County-Minnesota residents (January 1980-December 2019) using the Rochester Epidemiology Project medical records linkage system.

There were 20 sIBM patients (10 clinicopathologically-defined, 9 clinically-defined and 1 probable) and 1 patient with clinicopathologically-defined sIBM but symptom onset at age 43. The median age at symptom onset was 67 years (range: 43-86). The median time from symptom onset-to-diagnosis was 2 years (range <1 to 10 years). The prevalence of sIBM was 27.82, 22.39 and 18.20 per 100,000 people ≥ 50 years old in 1990, 2000 and 2010, respectively. All patients eventually needed walking aids, and 42% required a wheelchair. The median time from symptom onset-to-using cane or wheelchair was 5 (range, 1-13) and 10.5 (range, 1-29) years, respectively. Half of the patients required walking aids by 6 years from symptom onset. Eleven patients received immunomodulatory therapy without improvement. Overall survival was shorter in sIBM compared to the general population [84.1(95%CI: 78-88.4) versus 87.5 (95%CI: 85.2-89.5) years, P=0.03]. Thirteen patients died; death was sIBM-related in 9 (3 aspiration pneumonia, 4 neuromuscular respiratory failure and 2 unspecified sIBM complication). Patients with dysphagia had a higher mortality rate (P=0.05) than those without.
Prevalence of sIBM in Olmsted County is the highest reported to date. sIBM patients have a slightly shorter life expectancy compared to controls, especially in those with dysphagia.
Authors/Disclosures
Shahar Shelly, MD (Rambam Medical Center)
PRESENTER 		Dr. Shelly has or had stock in Remepy.
Michelle M. Mielke, PhD (Wake Forest University School of Medicine) Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for LabCorp. Dr. Mielke has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Siemens Healthineers. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sunbird Bio. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eisai. Dr. Mielke has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Roche. Dr. Mielke has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novo Nordisk.
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.
No disclosure on file
Elie Naddaf, MD (Mayo Clinic) Dr. Naddaf has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Expert Connect. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Klick, Inc. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for WebMD. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson and Johnson. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arcellx. The institution of Dr. Naddaf has received research support from NIAMS. The institution of Dr. Naddaf has received research support from Fulcrum therapeutics. The institution of Dr. Naddaf has received research support from Abcuro. The institution of Dr. Naddaf has received research support from Cabaletta . The institution of Dr. Naddaf has received research support from Arcellx.
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.