Newton Julia L, Hudson Mark, Tachtatzis Phaedra, Sutcliffe Kathryn, Pairman Jessie, Burt Jennifer A, Jones David E J
Liver Research Group, Institute of Cellular Medicine, Newcastle University, The Medical School, Framlington Place, Newcastle-upon-Tyne, UK.
Hepatology. 2007 Jun;45(6):1496-505. doi: 10.1002/hep.21609.
Patients with primary biliary cirrhosis (PBC) frequently experience significant fatigue thought to result from as-yet-unidentified central nervous system (CNS)-mediated processes. Pilot studies have suggested that autonomic dysfunction is a frequent occurrence in PBC and may contribute to the pathogenesis of this fatigue. The degree to which autonomic dysfunction affects the PBC population as a whole, and its interrelationship with other symptoms experienced by PBC patients remains unstudied. In this study, we used a geographically defined, fully representative PBC patient cohort to study the prevalence of symptoms of autonomic dysfunction and its relationship with other symptoms of PBC. Symptoms of cardiovascular autonomic dysfunction (as assessed using the Orthostatic Grading Scale [OGS]) were significantly more frequently reported and significantly more severe in PBC patients than in both matched normal controls (40% versus 6% with moderate or worse orthostasis (P < .0001), mean OGS score 3.2 +/- 3.4 versus 1.3 +/- 1.9, P < .005) and in patients with primary sclerosing cholangitis and in severity were independently associated with severity of fatigue and cognitive symptoms (both r2 = 0.3, P < .0001). Thirteen of 20 patients with an OGS value > 4 (moderate severity and worse) had significant abnormality in autonomic regulation of blood pressure, which was identified on dynamic testing.
Symptoms suggestive of autonomic dysfunction frequently occur in PBC patients and reflect dysregulation of actual blood pressure. Autonomic dysfunction is independently associated with both fatigue and, importantly, symptoms of cognitive dysfunction, suggesting the potential for significant organic sequelae.
原发性胆汁性肝硬化（PBC）患者经常经历严重疲劳，认为这是由尚未明确的中枢神经系统（CNS）介导的过程所致。初步研究表明，自主神经功能障碍在PBC中很常见，可能导致这种疲劳的发病机制。自主神经功能障碍对整个PBC人群的影响程度及其与PBC患者经历的其他症状的相互关系尚未得到研究。在本研究中，我们使用了一个地理定义明确、具有充分代表性的PBC患者队列，以研究自主神经功能障碍症状的患病率及其与PBC其他症状的关系。心血管自主神经功能障碍的症状（使用直立分级量表[OGS]评估）在PBC患者中报告的频率明显更高，且明显比匹配的正常对照组（中度或更严重直立性低血压者分别为40%对6%，P<.0001，平均OGS评分3.2±3.4对1.3±1.9，P<.005）以及原发性硬化性胆管炎患者更严重，其严重程度与疲劳和认知症状的严重程度独立相关（r2均=0.3，P<.0001）。20例OGS值>4（中度严重及更严重）的患者中有13例在动态测试中发现血压自主调节存在明显异常。
提示自主神经功能障碍的症状在PBC患者中经常出现，反映了实际血压的调节异常。自主神经功能障碍与疲劳以及重要的认知功能障碍症状独立相关，提示可能存在严重的器质性后遗症。
