Department of Medicine, Division of Clinical Pharmacology and the Autonomic Dysfunction Center, Vanderbilt University School of Medicine, Nashville, TN 37212, USA.
Pharmacol Ther. 2012 Jun;134(3):279-86. doi: 10.1016/j.pharmthera.2011.05.009. Epub 2011 Jun 12.
The clinical picture of autonomic failure is characterized by severe and disabling orthostatic hypotension. These disorders can develop as a result of damage of central neural pathways or peripheral autonomic nerves, caused either by a primary autonomic neurodegenerative disorder or secondary to systemic illness. Treatment should be focused on decreasing pre-syncopal symptoms instead of achieving blood pressure goals. Non-pharmacologic strategies such as physical counter-maneuvers, dietary changes (i.e. high salt diet, rapid water drinking or compression garments) are the first line therapy. Affected patients should be screened for co-morbid conditions such as post-prandial hypotension and supine hypertension that can worsen orthostatic hypotension if not treated. If symptoms are not controlled with these conservative measures the next step is to start pharmacological agents; these interventions should be aimed at increasing intravascular volume either by promoting water and salt retention (fludrocortisone) or by increasing red blood cell mass when anemia is present (recombinant erythropoietin). When pressor agents are needed, direct pressor agents (midodrine) or agents that potentiate sympathetic activity (atomoxetine, yohimbine, pyridostigmine) can be used. It is preferable to use short-acting pressor agents that can be taken on as needed basis in preparation for upright activities.
自主神经衰竭的临床表现为严重且使人致残的直立性低血压。这些疾病可由中枢神经通路或外周自主神经损伤引起，病因既有原发性自主神经退行性疾病，也有系统性疾病。治疗应侧重于减少晕厥前症状，而不是达到血压目标。非药物治疗策略，如身体对抗动作、饮食改变（即高盐饮食、快速饮水或压缩服装）是一线治疗。受影响的患者应筛查并存疾病，如餐后低血压和仰卧位高血压，如果不治疗，这些疾病可能会加重直立性低血压。如果这些保守措施不能控制症状，则下一步是开始使用药物治疗；这些干预措施旨在通过促进水和盐潴留（氟氢可的松）或在存在贫血时增加红细胞量（重组促红细胞生成素）来增加血管内体积。当需要升压药物时，可以使用直接升压药物（米多君）或增强交感神经活性的药物（托莫西汀、育亨宾、吡啶斯的明）。最好使用可按需服用的短效升压药物，以便在准备直立活动时使用。
