Patients with systemic sclerosis (SSc) frequently report muscle weakness. The reported prevalence of muscle involvement in SSc varies largely. There is no consensus whether or not an inflammatory myopathy in SSc should be merely considered as an organ complication of SSc, or as SSc-myositis overlap. Muscle weakness due to direct involvement of skeletal muscles in the disease process needs to be differentiated from myopathy secondary to disuse (fatigue, articular disease, cardiopulmonary involvement), kachectic atrophy, or from medications (statins, antimalarials etc.). Elevated muscle enzymes in patients’ serum may be only moderately elevated, or be completely within normal limits. Myopathy may be demonstrated by electromyography, skeletal muscle biopsy or magnetic resonance imaging. Blood oxygenation dependent (BOLD) MRI has demonstrated impaired skeletal muscle microcirculation.
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