Background Myopathy/myositis is associated with more severe systemic scleroderma (SSc).
Objectives The aim of this study was to determine such clinical information from the Canadian Scleroderma Research Group database (CSRG).
Methods Data from the CSRG are collected annually on SSc patients. Two surrogate markers for myopathy used in this study were elevated creatine kinase (CK) and physician-reported history of myopathy/myositis of the patients. Comparisons were made between those with and without myopathy/myositis to determine the strongest associations with this complication; overall, in lcSSc and dcSSc and in early dcSSc subset. Survival with and without myopathy/myositis was determined.
Results The study included 1143 patients with mean of 8 years of duration. Elevated CK occurred in 5.6%; 9.7% had a history of inflammatory myositis or myopathy according to physician; 5.7% had proximal muscle weakness. Those with elevated CK compared to remainder were more likely to be male (24.5% in elevated CK vs. 12.6% in normal CK; p<0.013), younger (51.93 vs. 56.07 years, p<0.045); have dcSSc, (40.4% vs. 37.9%; p<0.002), physician-reported history of myositis/myopathy (45.3% vs. 8.5%, p<0.000), tendon friction rubs (30.0% vs. 13.4%; p<0.001), FVC <70% (23.9% vs. 13.1%; p<0.039), RNP antibody (12.0% vs. 5.0%, p<0.032), Topo1 antibody (26.0% vs. 14.4%, p<0.026), higher skin scores (MRSS 16.14 vs. 9.81; p<0.000), and higher HAQ score (0.98 vs. 0.79; p<0.011). When using logistic regression younger age male, dcSSc, early dcSSc, tendon friction rubs, higher MRSS, Topo1, RNP, PMScl, and FVC<70% were associated with elevated CK. Survival was less in those with myopathy/myositis or elevated CK (p=0.003 and p=0.025 respectively). Those with elevated CK had less survival (p=0.025). Results were similar when the other definition of myopathy/myositis.
Conclusions Myopathy/myositis has a worse prognosis with respect to function and other organ involvement (ILD) and survival.
Disclosure of Interest M. Jung: None Declared, M. Baron: None Declared, M. Hudson: None Declared, A. Bonner: None Declared, J. Pope Grant/Research support from: Research support from CIHR, Scleroderma Society of Canada, Scleroderma Society of Ontario
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