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Distinct MR features in scleroderma associated myopathy

  • Magnetic Resonance Imaging
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Abstract

Objective

To compare the MRI features in patients with fibrosing [FM] versus non-fibrosing [NFM] systemic sclerosis [SSc]-associated myopathy.

Methods

10 patients with FM and 14 with NFM underwent bilateral thigh MRI [T1-weighted, STIR and DW/ADC mapping]. Three observers, blinded to histology evaluated 36 muscles per patient for presence of intramuscular edema, fascial edema, fatty replacement and atrophy and measured ADC values. Fisher's exact test and student’s t-test were used to compare MRI findings of FM [endomysial/ perimysial fibrosis] and NFM [necrosis/inflammation] on histology.

Results

Intramuscular edema [p < 0.0001] and fascial edema [p = 0.07] were more common in FM. On DWI, elevated intramuscular signal was more common in FM, [low b-value: p < 0.0001 and high b-value: p < 0.0001]. On T1, NFM exhibited more fatty replacement [p =  < 0.0001] and atrophy [p =  < 0.0001].

Conclusions

Intramuscular and fascial edema on MRI are more common in SSc-associated FM, while markers of chronic muscle damage are more often associated with NFM.

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Author notes

  1. Shivani Ahlawat and Julie Paik These authors contributed equally to the work.

Authors and Affiliations

  1. The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA

    Shivani Ahlawat, Elias S. Paris, Parvinder Sujlana & Laura M. Fayad

  2. University of Zurich, Raemistrasse 71, 8006, Zurich, Switzerland

    Filippo Del Grande

  3. Department of Radiology, Ospedale Regionale Di Lugano, Via Tesserete 46, 6900, Lugano, Switzerland

    Filippo Del Grande

  4. Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Julie Paik

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  1. Shivani Ahlawat
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  2. Julie Paik
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Corresponding author

Correspondence to Shivani Ahlawat.

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Conflict of interest

Shivani Ahlawat has received support from Department of Defense 2018–2023 and Pfizer (2017–2018). Laura M. Fayad has received support from GERRAF 2008–10 and Siemens 2011–12. Julie Paik has received supported in part by Doris Duke Early Clinician Investigator Award, Rheumatology Research Foundation, K Bridge Award, and Stauralaukis Discovery Award. The remaining authors have no conflicts of interest.

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This retrospective observational study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Human Investigation Committee (IRB) of Johns Hopkins University approved this study.

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Ahlawat, S., Paik, J., Del Grande, F. et al. Distinct MR features in scleroderma associated myopathy. Radiol med 126, 707–716 (2021). https://doi.org/10.1007/s11547-020-01317-5

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  • DOI: https://doi.org/10.1007/s11547-020-01317-5

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