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Abstract
Background and Objectives Limited data suggest that quantitative MRI (qMRI) measures have potential to be used as trial outcome measures in sporadic inclusion body myositis (sIBM) and as a noninvasive assessment tool to study sIBM muscle pathologic processes. Our aim was to evaluate changes in muscle structure and composition using a comprehensive multiparameter set of qMRI measures and to assess construct validity and responsiveness of qMRI measures in people with sIBM.
Methods This was a prospective observational cohort study with assessments at baseline (n = 30) and 1 year (n = 26). qMRI assessments include thigh muscle volume (TMV), inter/intramuscular adipose tissue (IMAT), muscle fat fraction (FF), muscle inflammation (T2 relaxation time), IMAT from T2* relaxation (T2*-IMAT), intermuscular connective tissue from T2* relaxation (T2*-IMCT), and muscle macromolecular structure from the magnetization transfer ratio (MTR). Physical performance assessments include sIBM Physical Functioning Assessment (sIFA), 6-minute walk distance, and quantitative muscle testing of the quadriceps. Correlations were assessed using the Spearman correlation coefficient. Responsiveness was assessed using the standardized response mean (SRM).
Results After 1 year, we observed a reduction in TMV (6.8%, p < 0.001) and muscle T2 (6.7%, p = 0.035), an increase in IMAT (9.7%, p < 0.001), FF (11.2%, p = 0.030), connective tissue (22%, p = 0.995), and T2*-IMAT (24%, p < 0.001), and alteration in muscle macromolecular structure (ΔMTR = −26%, p = 0.002). A decrease in muscle T2 correlated with an increase in T2*-IMAT (r = −0.47, p = 0.008). Deposition of connective tissue and IMAT correlated with deterioration in sIFA (r = 0.38, p = 0.032; r = 0.34, p = 0.048; respectively), whereas a decrease in TMV correlated with a decrease in quantitative muscle testing (r = 0.36, p = 0.035). The most responsive qMRI measures were T2*-IMAT (SRM = 1.50), TMV (SRM = −1.23), IMAT (SRM = 1.20), MTR (SRM = −0.83), and T2 relaxation time (SRM = −0.65).
Discussion Progressive deterioration in muscle quality measured by qMRI is associated with a decline in physical performance. Inflammation may play a role in triggering fat infiltration into muscle. qMRI provides valid and responsive measures that might prove valuable in sIBM experimental trials and assessment of muscle pathologic processes.
Classification of Evidence This study provides Class I evidence that qMRI outcome measures are associated with physical performance measures in patients with sIBM.
Glossary
- 2D=
- 2 dimensional;
- 3D=
- 3 dimensional;
- 6MWD=
- 6-minute walking distance;
- ActRII=
- activin type II receptor;
- BFL=
- biceps femoris long head;
- CV=
- coefficient of variation;
- FF=
- fat fraction;
- FOV=
- field of view;
- GRA=
- gracilis;
- IMAT=
- inter/intramuscular adipose tissue;
- MTC=
- magnetization transfer contrast;
- MTR=
- magnetization transfer ratio;
- qMRI=
- quantitative MRI;
- QMT=
- quantitative muscle testing;
- SAR=
- sartorius;
- SCAT=
- subcutaneous adipose tissue;
- sIBM=
- sporadic inclusion body myositis;
- sIFA=
- sIBM Physical Functioning Assessment;
- SM=
- semimembranosus;
- SPGR=
- spoiled gradient recalled echo;
- SRM=
- standardized response mean;
- ST=
- semitendinosus;
- T2*-IMAT=
- IMAT from T2* relaxation;
- T2*-IMCT=
- intermuscular connective tissue from T2* relaxation;
- TE=
- echo time;
- TFL=
- tensor fascia latae;
- TMV=
- thigh muscle volume;
- TR=
- repetition time;
- VLI=
- vastus lateralis and intermedius;
- VM=
- vastus medialis
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
Solicited and externally peer reviewed. The handling editor was Anthony Amato, MD, FAAN.
Editorial, page 361
Class of Evidence: NPub.org/coe
- Received September 6, 2021.
- Accepted in final form April 11, 2022.
- © 2022 American Academy of Neurology
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