Di Battista Marco, Wasson Christopher W, Alcacer-Pitarch Begonya, Del Galdo Francesco
Rheumatology Unit, University of Pisa, Pisa, Italy; Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
Semin Arthritis Rheum. 2023 Dec;63:152268. doi: 10.1016/j.semarthrit.2023.152268. Epub 2023 Sep 26.
Over the years several lines of evidence have implied a pathological involvement of autonomic nervous system (ANS) in systemic sclerosis (SSc). However, the relationship between autonomic dysfunction and SSc is not yet fully understood. The aims of this scoping review were to map the research done in this field and inform future research to investigate pathogenic hypotheses of ANS involvement.
We performed a scoping review of publications collected through a literature search of MEDLINE and Web of Science databases, looking for dysautonomia in SSc. We included original data from papers that addressed ANS involvement in SSc regarding pathogenesis, clinical presentation and diagnostic tools.
467 papers were identified, 109 studies were selected to be included in the present review, reporting data from a total of 2742 SSc patients. Cardiovascular system was the most extensively investigated, assessing heart rate variability with 24 h HolterECG or Ewing's autonomic tests. Important signs of dysautonomia were also found in digital vasculopathy, gastrointestinal system and SSc skin, assessed both with non-invasive techniques and histologically. Research hypotheses mainly regarding the relationship between sympathetic system - ischemia and the role of neurotrophins were then developed and discussed.
We described the currently available evidence on pathogenesis, clinical presentation and diagnostic assessment of dysautonomia in SSc patients. A strong influence of ANS deregulation on SSc clearly emerges from the literature. Future research is warranted to clarify the mechanisms and timing of autonomic dysfunction in SSc.
多年来，多条证据表明自主神经系统（ANS）参与系统性硬化症（SSc）的病理过程。然而，自主神经功能障碍与SSc之间的关系尚未完全明确。本综述的目的是梳理该领域的研究情况，并为未来研究提供信息，以探究ANS参与的致病假说。
我们对通过检索MEDLINE和科学网数据库收集的出版物进行了综述，查找SSc中的自主神经功能障碍。我们纳入了涉及ANS参与SSc发病机制、临床表现和诊断工具的论文的原始数据。
共识别出467篇论文，选择109项研究纳入本综述，报告了总共2742例SSc患者的数据。心血管系统是研究最广泛的，通过24小时动态心电图或尤因自主神经测试评估心率变异性。在数字血管病变、胃肠道系统和SSc皮肤中也发现了自主神经功能障碍的重要迹象，采用非侵入性技术和组织学方法进行评估。随后提出并讨论了主要关于交感神经系统 - 缺血之间关系以及神经营养因子作用的研究假说。
我们描述了目前关于SSc患者自主神经功能障碍发病机制、临床表现和诊断评估的现有证据。文献中明确显示ANS失调对SSc有很大影响。有必要进行未来研究以阐明SSc中自主神经功能障碍的机制和时间。
