Abstract Details

Title Increased Mortality and Higher Frequency of Hematologic Malignancies in Inclusion Body Myositis: A Population-Based Case-Control Study

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 110

Objective To evaluate the mortality and comorbidities associated with inclusion body myositis (IBM), in a population-based case-control study.

Background

Given the poorly understood pathogenesis of IBM, several studies evaluated the association with various autoimmune, infectious, neurodegenerative and neoplastic disorders. Furthermore, the effect of IBM on longevity is not well established.

Design/Methods We utilized the expanded Rochester Epidemiology Project, which became available in 2010, and includes Olmsted and 26 other counties in Minnesota and Wisconsin. We identified 50 patients with IBM (ENMC criteria) seen between 2010 and 2018, 68 age/sex-matched other idiopathic inflammatory myopathy (IIM) patients, and 294 age/sex-matched controls. We compared the frequency of various associated conditions (searched by ICD codes), and the mortality between groups.

Results In IBM group, mean age at index was 74 years, and 58% of patients were male. Only 9 (18%) IBM patients were treated with an oral immunosuppressant. The frequency of neurodegenerative disorders (i.e. dementia, Parkinson’s disease) was not different between groups, whereas peripheral neuropathy was more common in IBM patients (44% versus 37% in IIIM and 27% in controls, p=0.001). Hematologic malignancies, and not solid cancers, were more common in IBM (10%) and IIM (8.8%), compared to controls (2.7%) (p=0.0124). IBM was associated with increased mortality compared to IIM (odds ratio of 3.027, p=0.004), and to controls (odds ratio of 2.793, p=0.001), with a mean age at death of 79 in IBM group, 76 in IIM group, and 84 in controls. The top 3 causes of death in IBM group were: respiratory failure or pneumonia (44%), cancer (15%), and advanced age and medical comorbidities (15%).

Conclusions IBM patients have increased mortality and IBM-related complications are the most common cause of death in this group. Peripheral neuropathy, but not neurodegenerative disorders, and hematologic malignancies, but not solid tumors, occur more frequently in IBM patients than in the general population.

Authors/Disclosures
Elie Naddaf, MD (Mayo Clinic) PRESENTER	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.
Shahar Shelly, MD (Rambam Medical Center)	Dr. Shelly has or had stock in Remepy.
Jayawant N. Mandrekar, PhD	Dr. Mandrekar has nothing to disclose.
	No disclosure on file
E. M. Hoffman, DO, PhD (Mayo Clinic, Neurology)	Dr. Hoffman has nothing to disclose.
	No disclosure on file
Teerin Liewluck, MD, F�鶹��ýӳ�� (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.

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