Carr Zyad J, Klick John, McDowell Brittany J, Charchaflieh Jean G, Karamchandani Kunal
Department of Anesthesiology, Yale New Haven Hospital, New Haven, CT 06510 USA.
Yale University School of Medicine, New Haven, CT 06510 USA.
Curr Anesthesiol Rep. 2020;10(4):512-521. doi: 10.1007/s40140-020-00411-8. Epub 2020 Aug 29.
Systemic sclerosis or scleroderma (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis that results in progressive end-organ dysfunction and decreased survival. SSc results in significant challenges for the practicing anesthesiologist due to its rarity, multi-system involvement, and limited evidence-based guidance for optimal perioperative care. In this update, we briefly discuss the recent evidence on the pathophysiology and current management of SSc, review the anesthesia-related literature, and extrapolate these observations into an optimal perioperative strategy for the care of SSc patients.
Evidence shows that patients with SSc demonstrate an increased risk for perioperative myocardial infarction, high rates of interstitial lung disease, pulmonary arterial hypertension, neurological disease, gastric dysmotility disorders, and challenging airway management, all findings that may result in suboptimal perioperative outcomes.
Advances in SSc medical management have resulted in improved survival, likely increasing the number of patients who will be exposed to perioperative care. Optimal perioperative management and risk stratification should expand beyond the well-described airway challenges and consider numerous systemic manifestations of systemic sclerosis such as pulmonary arterial hypertension, interstitial lung disease, and cardiac sequelae.
系统性硬化症（SSc），又称硬皮病，是一种系统性、免疫介导的疾病，其特征为皮肤和器官出现异常纤维化，导致进行性终末器官功能障碍和生存率降低。由于SSc较为罕见、累及多系统且缺乏关于最佳围手术期护理的循证指南，给执业麻醉医师带来了重大挑战。在本次更新中，我们简要讨论了关于SSc病理生理学和当前治疗的最新证据，回顾了与麻醉相关的文献，并将这些观察结果推断为针对SSc患者护理的最佳围手术期策略。
证据表明，SSc患者围手术期心肌梗死风险增加，间质性肺疾病、肺动脉高压、神经疾病、胃动力障碍发生率高，气道管理具有挑战性，所有这些发现都可能导致围手术期结果不理想。
SSc医疗管理的进展提高了生存率，可能会增加接受围手术期护理的患者数量。最佳围手术期管理和风险分层应超越已充分描述的气道挑战，考虑系统性硬化症的多种全身表现，如肺动脉高压、间质性肺疾病和心脏后遗症。
