Comparative Evaluation of Pulmonary Function Tests in IBM based on serology to NT5c1A Antibodies


Topic:

Other

Poster Number: M241

Author(s):

Karina Bjazevic, BS BA, University of California, Irvine, Tahseen Mozaffar, MD, UCI Health, Aziz Shaibani, MD, Nerve and Muscle Center of Texas, Conrad Weihl, MD, PhD, Washington University in Saint Louis, Thomas Lloyd, MD, PhD, Johns Hopkins University, Colin Quinn, MD, University of Pennsylvania, Mazen Dimachkie, MD, University of Kansas Medical Center, Olimpia Carbunar, MD, University of Miami Health Systems, Miriam Freimer, MD, Ohio State University, Namita Goyal, MD, UCI Health, Marie Wencel, BS, UCI Health

OBJECTIVE:
To evaluate the relationship between pulmonary function tests (PFT) results, including sitting and supine FVC, MIP, and MEP, and seropositivity for NT5c1A antibody in IBM subjects.
BACKGROUND:
Inclusion body myositis (IBM) is the most common acquired muscle disease found in individuals over the age of 40, with a predominance in males. Affected individuals experience a slowly progressive and asymmetric weakness that affects mobility and daily activities. Respiratory failure is a common outcome for IBM patients. Previous analysis in small cohorts of IBM patients has shown subjects who are seropositive for NT5c1A antibody demonstrate significantly lower results in MIP and FVC testing, thus predicting more severe respiratory involvement. However, further analysis on pulmonary function and seropositivity for NT5c1A antibody in a larger cohort is required. INSPIRE-IBM is a prospective natural history study of 150 IBM patients, across thirteen US sites. One of the aims of this study is to explore differences in pulmonary function relative to the serological biomarkers, and document the natural history of the pulmonary functions over a two year period.
DESIGN/METHODS:
An analysis of baseline PFT data, including FVC, MIP, and MEP, within a cohort of 150 IBM patients from INSPIRE-IBM will be conducted. Additionally, the association between these pulmonary function tests and the seropositivity for NT5c1A antibody will be explored.
RESULTS/CONCLUSION:
Analysis will take place after the closure of enrollment in January 2024 and the completion of baseline visits for all subjects. Baseline data will be presented.