Correlations of disease severity outcome measures in inclusion body myositis.

This study aimed to evaluate the correlation between various outcome measures used to assess disease severity and progression in inclusion body myositis (IBM) clinical trials. A cross-sectional study, involving 51 IBM patients meeting the European Neuromuscular Center (ENMC) 2011 criteria for clinically defined or probable IBM, was completed at the University of California, Irvine. Clinical details, demographic data, and functional data including timed get up (TGU), manual muscle testing, hand grip, pinch dynamometry, as well as IBM functional rating scale (IBMFRS), modified Rankin score, forced vital capacity (FVC), and modified ocular bulbar facial respiratory scale (mOBFRS) were collected on all patients. Descriptive statistics and Pearson's r correlation were performed to analyze the data. Age of the patient did not correlate with any of the outcome measures. Greater severity of IBMFRS scores correlated with longer disease duration as well as greater severity for FVC, strength outcomes, TGU, modified Rankin, and mOBFRS. Additionally, TGU strongly correlated with muscle strength measurements, modified Rankin, and mOBFRS. mOBFRS moderately correlated with IBMFRS, muscle strength, FVC, TGU and modified Rankin score. We demonstrate moderate to strong correlations among the disease severity outcome measures in this study.
Competing Interests: Declaration of Competing Interest S.A.G. is an inventor of intellectual property related to myositis diagnostics and therapeutics, owned and managed by Brigham and Women's Hospital (BWH); he is a founder of Abcuro, Inc. Partners HealthCare, the owner of BWH, and S.A.G. have financial interests in Abcuro. The financial interests were reviewed and managed in accordance with the conflict-of-interest policies of Partners HealthCare. A.M.P. was supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant UL1 TR001414. S.A.V. was supported by PHS grants R01NS120060, R21NS114918, and KL2TR001416. T.M. was supported by PHS grants R01AR078340, UL1TR001414 and U24NS107210. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH. The rest of the authors report no relevant disclosures.
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