INTRODUCTION Autonomic dysfunction, an early symptom of transthyretin amyloidosis (ATTR amyloidosis), requires investigations not readily available in many clinics. Although monitoring of orthostatic hypotension (OH) will not be a substitute for more specialized tests, it can add important information about initiation of dysautonomia. The aim of this study was to investigate whether simple blood pressure (BP) monitoring may be a useful tool for evaluation of disease progression and an early sign of autonomic dysfunction. METHODS BP and OH data were from subjects enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS). Characteristics associated with changes in BP and orthostatic difference were identified by regression analyses. RESULTS OH tended to be present relatively early in the course of disease and was more common at enrollment (11.7%) than either diarrhea (2.4%) or unintentional weight loss (3.1%). In subjects with OH at enrollment, progressive increase in systolic and diastolic orthostatic difference was observed. OH was also associated with significantly worse quality of life. DISCUSSION BP variability is a useful tool for assessing disease onset and severity in ATTR amyloidosis, particularly in patients with OH. Trial registration ClinicalTrials.gov: NCT00628745.

中文翻译：

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血压和体位性低血压作为转甲状腺素蛋白淀粉样变性结果调查 (THAOS) 患者自主神经功能障碍的量度

引言自主神经功能障碍是甲状腺素运载蛋白淀粉样变性（ATTR 淀粉样变性）的早期症状，需要在许多诊所进行调查。尽管监测直立性低血压 (OH) 并不能替代更专业的测试，但它可以增加关于自主神经功能障碍开始的重要信息。本研究的目的是调查简单的血压 (BP) 监测是否可能是评估疾病进展和自主神经功能障碍的早期迹象的有用工具。方法 BP 和 OH 数据来自参加甲状腺素运载蛋白淀粉样变性结果调查 (THAOS) 的受试者。通过回归分析确定了与血压变化和体位差异相关的特征。结果 OH 在疾病过程中相对较早出现，并且在入组时 (11.7%) 比腹泻 (2.4%) 或无意体重减轻 (3.1%) 更常见。在入组时患有 OH 的受试者中，观察到收缩压和舒张压直立性差异逐渐增加。OH 也与显着更差的生活质量有关。讨论 血压变异性是评估 ATTR 淀粉样变性的发病和严重程度的有用工具，特别是在 OH 患者中。试验注册 ClinicalTrials.gov：NCT00628745。讨论 血压变异性是评估 ATTR 淀粉样变性的发病和严重程度的有用工具，特别是在 OH 患者中。试验注册 ClinicalTrials.gov：NCT00628745。讨论 血压变异性是评估 ATTR 淀粉样变性的发病和严重程度的有用工具，特别是在 OH 患者中。试验注册 ClinicalTrials.gov：NCT00628745。