Cuevas Sandra, Mayer Erik, Hughes Michael, Adler Brittany L, McMahan Zsuzsanna H
University of Puerto Rico School of Medicine, San Juan, Puerto Rico.
McMaster University, Hamilton, ON, Canada.
J Scleroderma Relat Disord. 2025 Jan 8:23971983241308050. doi: 10.1177/23971983241308050.
Autonomic dysfunction is a common and early complication among patients with systemic sclerosis, suggesting that it may play a role in the pathogenesis of the disease and be a potential target for therapeutic interventions. Although the true prevalence of autonomic dysfunction among patients with systemic sclerosis is still unclear, it is estimated that as many as 80% of patients may be affected. Autonomic dysfunction may lead to widespread multi-organ dysfunction through its effects on the cardiovascular system, gastrointestinal tract, urinary tract, sweat and salivary glands, and pupils. Early identification of systemic sclerosis associated with dysautonomia may guide prompt diagnosis in this complex patient population and lay the groundwork for future research in this area. Furthermore, the current landscape of targeted interventions for autonomic dysfunction is rapidly expanding; therefore, prioritizing patients who may benefit from such interventions or candidates for related clinical trials is paramount. Our scoping review details timely updates in the extant literature, including findings from recent studies on autonomic dysfunction in systemic sclerosis, and integrates these updates to identify critical gaps in the field.
自主神经功能障碍是系统性硬化症患者常见的早期并发症，这表明它可能在该疾病的发病机制中起作用，并且是治疗干预的潜在靶点。尽管系统性硬化症患者中自主神经功能障碍的真实患病率仍不清楚，但据估计，多达80%的患者可能受到影响。自主神经功能障碍可能通过对心血管系统、胃肠道、泌尿系统、汗腺和唾液腺以及瞳孔的影响，导致广泛的多器官功能障碍。早期识别与自主神经功能障碍相关的系统性硬化症，可能有助于在这一复杂患者群体中进行及时诊断，并为该领域的未来研究奠定基础。此外，目前针对自主神经功能障碍的靶向干预格局正在迅速扩大；因此，优先考虑可能从此类干预中受益的患者或相关临床试验的候选者至关重要。我们的范围综述详细介绍了现有文献中的及时更新，包括最近关于系统性硬化症中自主神经功能障碍的研究结果，并整合这些更新以确定该领域的关键差距。
