Department of Rheumatology and Physiotherapy, "Grigore T. Popa" University of Medicine and Pharmacy, 16 University Street, 700115 Iasi, Romania.
Department of Pharmacology, "Grigore T. Popa" University of Medicine and Pharmacy, 16 University Street, 700115 Iasi, Romania.
Int J Mol Sci. 2022 Oct 9;23(19):12011. doi: 10.3390/ijms231912011.
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by heterogeneous changes involving numerous organs and systems. The currently available data indicate that muscle injury (both smooth and striated muscles) is widespread and leads to significant morbidity, either directly or indirectly. From the consequences of smooth muscle involvement in the tunica media of blood vessels or at the level of the digestive tract, to skeletal myopathy (which may be interpreted strictly in the context of SSc, or as an overlap with idiopathic inflammatory myopathies), muscular injury in scleroderma translates to a number of notable clinical manifestations. Heart involvement in SSc is heterogenous depending on the definition used in the various studies. The majority of SSc patients experience a silent form of cardiac disease. The present review summarizes certain important features of myocardial, as well as smooth and skeletal muscle involvement in SSc. Further research is needed to fully describe and understand the pathogenic pathways and the implications of muscle involvement in scleroderma.
系统性硬化症(SSc)是一种复杂的自身免疫性疾病,其特征为涉及众多器官和系统的异质性变化。目前的数据表明,肌肉损伤(平滑肌和横纹肌)广泛存在,并直接或间接导致显著的发病率。从血管中层的平滑肌受累或消化道的平滑肌受累的后果,到骨骼肌病(可能在 SSc 背景下严格解释,或与特发性炎性肌病重叠),硬皮病中的肌肉损伤可转化为多种显著的临床表现。根据在各种研究中使用的定义,SSc 中的心脏受累具有异质性。大多数 SSc 患者经历一种无症状的心脏疾病。本综述总结了 SSc 中心肌、平滑肌和骨骼肌受累的某些重要特征。需要进一步研究来充分描述和理解肌肉受累在硬皮病中的发病机制和影响。