To examine the possible relationship between cardiac and skeletal muscle disease in systemic sclerosis, we reviewed computerized records of 1095 consecutive patients with systemic sclerosis. One hundred eighty three (17%) had skeletal myopathy. Thirty-nine (21%) of the 183 fulfilled criteria for myocardial disease, compared with 90 (10%) of the 912 without myopathy (p < 0.0001.) Nineteen (10%) of the 183 had clinical CHF compared with 38 (4%) of the remainder (p < 0.002.) Fifteen (8%) of the patients with myopathy died of cardiac causes compared with 27 (3%) of the 912 without myopathy (p < 0.002.) Twenty-five patients with coexistent myopathy and myocardial disease, in the absence of other identifiable contributing causes, were identified. This group was characterized by a high incidence of cardiac conduction abnormalities (60%) and by the severity of the myocardial dysfunction and arrhythmias, both atrial and ventricular that they experienced. Eighteen of these 25 patients died; 12 (67%) died suddenly. Eight of the 18 (44%) had intractable CHF, which directly contributed to their deaths. Myocardial fibrosis was the predominant histologic abnormality at autopsy. However, autopsy of a patient who died in the context of acute "myocarditis" showed severe myocytolysis with contraction band necrosis but without inflammation or fibrosis; this is consistent with possible ischemically mediated injury. We conclude that skeletal and cardiac muscle disease in systemic sclerosis are associated. Patients with myopathy are at increased risk for CHF, sustained symptomatic arrhythmias, and cardiac death, particularly sudden death.
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http://dx.doi.org/10.1016/0002-8703(93)90075-k | DOI Listing |
Objective: Scleroderma-associated autoantibodies (SSc-Abs) are specific in participants (pts) with systemic sclerosis and are associated with organ involvement. Our objective was to assess the influence of baseline SSc-Abs on the trajectories of the clinical outcome assessments (COAs) in a phase III randomized controlled trial.
Methods: We used data on both the groups who received placebo (Pbo) and tocilizumab from the focuSSced trial.
J Scleroderma Relat Disord
January 2025
Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, USA.
Background: Pain is a prevalent symptom of systemic sclerosis. While previous studies have demonstrated a correlation between higher pain intensity and lower physical function in individuals with systemic sclerosis, the potentially moderating effect of psychosocial factors on the association has yet to be explored.
Methods: This cross-sectional study used data from a fatigue self-management trial for adults with systemic sclerosis.
J Scleroderma Relat Disord
January 2025
Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Cardiovascular complications are observed in up to one-third of patients with systemic sclerosis (SSc). Early identification and management of SSc-associated primary cardiac disease is often challenging, given the complex disease pathophysiology, significant variability in clinical presentation, and scarce disease-modifying therapeutics. Here, we review the molecular mechanisms involved in SSc-associated cardiac disease pathogenesis, novel diagnostic tools and emerging therapies.
View Article and Find Full Text PDFAutoimmun Rev
January 2025
The Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan, China; National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Changsha, Hunan, China; Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Changsha, Hunan, China; Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Changsha, Hunan, China. Electronic address:
Autoimmune diseases (ADs) are a group of complex, chronic conditions characterized by disturbance of immune tolerance, with examples including systemic lupus erythematosus, rheumatoid arthritis, multiple sclerosis, and psoriasis. These diseases have unclear pathogenesis, and traditional therapeutic approaches remain limited. However, advances in high-throughput histology technology and scientific discoveries have led to the identification of various pathogenic factors contributing to ADs.
View Article and Find Full Text PDFAnn Med
December 2025
Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
Background: Calcinosis cutis of hands can progress and impair hand function in systemic sclerosis (SSc). Understanding the natural disease and comprehensive management is crucial.
Objective: To examine clinical course and identify risk factors associated with progressive calcinosis cutis in early SSc.
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