目的:关于内源性库欣综合征(CS)的文献主要集中在诊断的挑战,亚型,和治疗。糖皮质激素戒断综合征(GWS)的神秘现象,由于CS治疗后皮质醇暴露迅速减少,很少讨论,但也很难管理。我们强调了将患者从GWS和肾上腺功能不全导航到下丘脑-垂体-肾上腺(HPA)轴恢复的临床方法。
方法:我们回顾了有关GWS的发病机制及其临床表现的文献。我们提供糖皮质激素给药和减量的策略,HPA轴测试,以及GWS症状管理的药物治疗和辅助治疗。
结果:GWS很难与肾上腺功能不全和CS复发区分,这使得糖皮质激素给药和逐渐减少的方案复杂化。监测HPA轴恢复需要临床和生化评估。最重要的干预措施是向患者保证GWS症状预示CS持续缓解的良好预后。和GWS通常在HPA轴恢复时解析。GWS也发生在CS的医疗管理过程中,和逐渐的剂量滴定,主要基于症状是至关重要的,以保持坚持和最终实现疾病控制。肌病和神经认知功能障碍可能是CS的慢性并发症,无法完全恢复。
结论:由于数据有限,尚未制定GWS管理指南.然而,本文提供了从已发表的文献以及专家意见和经验中得出的总体主题。未来的研究需要更好地了解GWS的病理生理学,以指导更有针对性的优化治疗。
OBJECTIVE: Literature regarding endogenous Cushing syndrome (CS) largely focuses on the challenges of diagnosis, subtyping, and treatment. The enigmatic phenomenon of glucocorticoid withdrawal syndrome (GWS), due to rapid reduction in cortisol exposure following treatment of CS, is less commonly discussed but also difficult to manage. We highlight the clinical approach to navigating patients from GWS and adrenal insufficiency to full hypothalamic-pituitary-adrenal (HPA) axis recovery.
METHODS: We review the literature on the pathogenesis of GWS and its clinical presentation. We provide strategies for glucocorticoid dosing and tapering, HPA axis testing, as well as pharmacotherapy and ancillary treatments for GWS symptom management.
RESULTS: GWS can be difficult to differentiate from adrenal insufficiency and CS recurrence, which complicates glucocorticoid dosing and tapering regimens. Monitoring for HPA axis recovery requires both clinical and biochemical assessments. The most important intervention is reassurance to patients that GWS symptoms portend a favorable prognosis of sustained remission from CS, and GWS typically resolves as the HPA axis recovers. GWS also occurs during medical management of CS, and gradual dose titration based primarily on symptoms is essential to maintain adherence and to eventually achieve disease control. Myopathy and neurocognitive dysfunction can be chronic complications of CS that do not completely recover.
CONCLUSIONS: Due to limited data, no guidelines have been developed for management of GWS. Nevertheless, this article provides overarching themes derived from published literature plus expert opinion and experience. Future studies are needed to better understand the pathophysiology of GWS to guide more targeted and optimal treatments.