Skeletal Muscle Involvement in Systemic Sclerosis

Independent Association of Severity of Muscle Weakness With Disability as Measured by the Health Assessment Questionnaire Disability Index in Scleroderma

“…Skeletal muscle disease in scleroderma is common, with a prevalence of 13-81% depending on the criteria used to define muscle involvement (1)(2)(3)(4)(5)(6)(7). Some studies have defined muscle disease by subjective proximal weakness only, while more rigorous investigations require electromyography and muscle biopsies to define muscle disease (8).…”

A descriptive and prognostic study of systemic sclerosis-associated myopathies

“…Bioclinical and electromyographic features of SSc-related myopathies are indistinguishable from those of polymyositis,6 although severe muscle weakness5 and high muscle enzyme levels34 seem less frequent. We did not observe any significant association between the bioclinical presentation of myopathy and muscle outcome upon multivariate analysis.…”

“…The prevalence of myopathies in SSc patients varies from 14% to 81%,34567 depending on the diagnostic criteria for muscle involvement. SSc-associated myopathies share numerous clinical, biological and electromyographic features with idiopathic inflammatory myopathies 7.…”

“…SSc-associated myopathies share numerous clinical, biological and electromyographic features with idiopathic inflammatory myopathies 7. In contrast, histological features are heterogeneous, and may include more specific aspects such as stigma of microangiopathy48 or increased fibrosis in the perimysium and epimysium 39…”

Significant Increase of Urinary Low-Sulfated Heparan-Sulfate-Related Protein in Patients with Severe Systemic Scleroderma

“…Inflammatory pro cesses in patients with myositis were noted by Winkelmann [16] as sclerodermatomyositis; the severity of this type of scleroderma as well as its good response to corti costeroids have been described [3,10,13,15]. Therefore, an increase in urinary LSHSrelated protein may reflect either the inflam mation or activated connective tissue metabolism [1] in severe SSD.…”