4.4 Article

Disease specific determinants of cardiopulmonary fitness in systemic sclerosis

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W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.semarthrit.2022.152137

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Systemic sclerosis; Cardiopulmonary exercise; Cardiac disease; Myositis; Physical function

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This study aimed to quantify the burden of exercise intolerance in systemic sclerosis (SSc) and explore the disease features contributing to impaired exercise capacity. The results showed significantly reduced exercise capacity in SSc patients compared to controls, with diffuse myocardial fibroinflammatory disease and skeletal muscle edema being significantly associated with reduced exercise tolerance. Left ventricular ejection fraction was not associated with impaired exercise capacity.
Objectives: We aimed to quantify the burden of exercise intolerance in systemic sclerosis (SSc) and explore the disease features that contribute to impaired exercise capacity (measured as peak oxygen uptake, peak VO2) to provide novel mechanistic insights into the causes of physical disability in SSc.Methods: Thirty-three SSc patients with no history of cardiac disease and no active myositis underwent cardiac and skeletal muscle MRI, transthoracic echocardiography, pulmonary function tests and cardiopulmonary ex-ercise testing (CPET). CPET results were compared to an age-, sex-, and weight-matched controls with no overt cardiopulmonary disease. Native T1 and T2-mapping sequences were used to quantify diffuse fibroinflammatory myocardial disease and qualitative assessment of skeletal muscle oedema was performed. The associations be-tween parameters of cardiorespiratory function and skeletal muscle abnormalities and peak VO2 were evaluated with linear regression analysis.Results: Exercise capacity was markedly impaired in SSc and significantly reduced when compared to control subjects (percent predicted peak VO2: 70% vs 98%, p < 0.01). Diffuse myocardial fibroinflammatory disease (p < 0.01) and skeletal muscle oedema (p = 0.01) were significantly associated with reduced exercise capacity. There was no association between impaired exercise capacity and left ventricular ejection fraction.Conclusion: SSc is associated with marked functional impairment that is not explained by commonly used pa-rameters of cardiac function such as left ventricular ejection fraction. Rather, only more sensitive measures of organ involvement are associated with impaired exercise tolerance. Our results show diffuse interstitial changes of the myocardium and skeletal muscle affect oxygen uptake and are important contributors to functional lim-itation in SSc.

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