ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 798

Predominant Fasciitis and Mild Intramuscular Edema on Muscle Magnetic Resonance Imaging in Scleroderma-Associated Myopathy

Julie J. Paik1, Shivani Ahlawat2, Filippo DelGrande3, Laura K. Hummers4, Fredrick M. Wigley4, Ami A. Shah4, Andrew Mammen5 and Laura Fayad6, 1Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 2Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, 3Johns Hopkins University, Baltimore, MD, 4Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 5National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 6Radiology, Johns Hopkins University, Baltimore, MD

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Date: Sunday, October 21, 2018

Title: Systemic Sclerosis and Related Disorders – Clinical Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Skeletal myopathy in systemic sclerosis is poorly defined. The spectrum of muscle histopathology in systemic sclerosis has been previously studied and shown to be heterogeneous. There has been little data to describe the imaging findings in scleroderma muscle disease. The purpose of this study is to determine whether there are unique muscle magnetic resonance imaging (MRI) features of scleroderma associated myopathy.

Methods:

This retrospective, cross-sectional study included 25 patients with SSc and biopsy proven fibrosing myopathy or inflammatory myopathy as previously described (1). These patients also had coronal and axial T1-weighted images and short tau inversion recovery (STIR images) of the thighs as part of routine clinical care. A small subset (n=15) also had axial diffusion weighted imaging (DWI) using 2 b-values (0 and 800 s/mm2) and apparent diffusion coefficient (ADC) mapping. Two musculoskeletal radiologists, blinded to histopathological finding, reviewed the MR studies in consensus and semi-quantitatively evaluated for the presence of intramuscular edema, fascial edema, fatty replacement and atrophy on all sequences using equidistant 4-point scoring system (0 = absence, 1 = mild, 2 = moderate, 3 = maximal) for 36 individual skeletal muscles in each patient. Pairwise comparisons for categorical variables between the fibrosing and non-fibrosing groups were made using χ2 test.

Results: There were 12 patients with fibrosing myopathy and 13 patients with an inflammatory myopathy with available MRI data. On STIR sequences, patients with a fibrosing myopathy more often had mild intramuscular (48% (170/355) vs. 36% (176/485), p=.0005) but more prominent fascial edema (16% (46/294) vs.9% (47/496), p=.0005) when compared with the inflammatory myopathy group. Whereas, on T1-weighted sequences, patients with an inflammatory myopathy more often had evidence of chronic muscle damage such as mild fatty replacement (36% (182/504) vs. 27% (82/306), p=0.008) and atrophy (44% (217/498) vs. 34% (104/306), p=0.005) when compared with a fibrosing myopathy. On DWI, patients with SSc-associated fibrosing myopathy more often had elevated signal for both low and high b-value images.

Conclusion:

Thigh muscle MRI may be a useful imaging biomarker to distinguish fibrosing myopathy from inflammatory myopathy in systemic sclerosis.

Reference

  1. Paik JJ, Wigley FM, Shah AA, Corse AM, Casciola-Rosen L, Hummers LK, et al. Fibrosing myopathy in systemic sclerosis associates with higher mortality. Arthritis Care Res 2017.
Disclosure: J. J. Paik, None; S. Ahlawat, None; F. DelGrande, None; L. K. Hummers, None; F. M. Wigley, None; A. A. Shah, Bristol-Myers Squibb, 5; A. Mammen, None; L. Fayad, None.

To cite this abstract in AMA style:

Paik JJ, Ahlawat S, DelGrande F, Hummers LK, Wigley FM, Shah AA, Mammen A, Fayad L. Predominant Fasciitis and Mild Intramuscular Edema on Muscle Magnetic Resonance Imaging in Scleroderma-Associated Myopathy [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/predominant-fasciitis-and-mild-intramuscular-edema-on-muscle-magnetic-resonance-imaging-in-scleroderma-associated-myopathy/. Accessed .

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