Abstract Details

Title Clinical Correlates of Variant KLRG1-expressing T cells in Inclusion Body Myositis

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P16 - Poster Session 16 (5:30 PM-6:30 PM)

Poster/Presentation
Number 1-003

Objective To correlate disease behavior (disease phenotype and severity) in inclusion body myositis (IBM) with variant killer cell lectin-like receptor G1 (KLRG1)- expressing T-lymphocytes in the circulating blood of these patients.

Background Recent advances has resurrected the implication of immune dysfunction as the pathogenic mechanism in IBM, including the identification of the circulating NT5c1A autoantibody, presence of cytotoxic T cell signature in gene expression studies, and identification of clonal highly differentiated “immunosenescent” cytotoxic CD8+T-cell effector cells in muscle and blood expressing KLRG1.

Design/Methods A cross-sectional study, involving 50 consecutive IBM patients meeting the ENMC 2011 criteria for clinically defined or probable IBM, is currently ongoing at the University of California, Irvine. The patients undergo serological testing for the NT5c1A antibodies and for a battery of cell differentiation (CD) markers in lymphocytes isolated from peripheral blood through multiplex flow cytometry, including CD8, CD28, CD57 and KLRG1. Clinical details, demographics, functional data (timed get up, manual muscle testing, hand grip, pinch grip, IBM functional rating scale, modified Rankin score, forced vital capacity, presence of dysphagia, use of assistive devices), and quality of life questionnaires are being collected on all patients.

Results The study is ongoing and will be completed in November 2019. The data will be analyzed to evaluate the presence and relationship of the atypical lymphocytes in circulating blood of IBM patients with disease phenotype, quality of life, functional disability and need for ambulatory devices in these patients.

Conclusions Our study will evaluate if increased frequency of KLRG1⁺CD8⁺cells correlate with markers of disease severity in IBM patients compared to those IBM patients who do not harbor these cells.

Authors/Disclosures
Namita Goyal, MD, F�鶹��ýӳ�� (University of California, Irvine) PRESENTER	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.
Steven A. Greenberg, MD (Brigham and Women's Hospital Neurology)	Dr. Greenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Abcuro, Inc.. Dr. Greenberg has stock in Abcuro. Dr. Greenberg has received intellectual property interests from a discovery or technology relating to health care.
Jonathan Cauchi, MD (University of New Mexico Hospital)	Dr. Cauchi has nothing to disclose.
	No disclosure on file
	No disclosure on file
Marie Wencel, CCRP (University of California, Irvine)	Ms. Wencel has nothing to disclose.
Tyler Irani, MD (Stanford University)	Dr. Irani has nothing to disclose.
	No disclosure on file
Tahseen Mozaffar, MD, F�鶹��ýӳ�� (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.

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