Dessein P H, Joffe B I, Metz R M, Millar D L, Lawson M, Stanwix A E
Department of Medicine, University of the Witwatersrand, Johannesburg, South Africa.
Am J Med. 1992 Aug;93(2):143-50. doi: 10.1016/0002-9343(92)90043-b.
This study was designed to assess the prevalence and nature of autonomic dysfunction (AD) in 34 patients with systemic sclerosis (SSc).
Patients were questioned for current symptoms possibly related to AD. Five noninvasive cardiovascular autonomic function tests and sequential plasma catecholamine estimations at rest, during standing, and during sustained handgrip were performed. Seven patients with manometrically documented esophageal involvement and high resting plasma adrenaline levels were treated with clonidine (75 to 375 micrograms/d). One month later, resting plasma catecholamine estimations and esophageal motility studies were repeated.
Autonomic testing revealed AD in each patient, while symptoms were experienced by 33 of them. Findings on two of the three heart rate tests and both blood pressure tests were significantly impaired as compared with those in 25 matched control subjects. Mean resting plasma adrenaline levels were 18 times higher than in 10 matched controls (p less than 0.001). Plasma catecholamine (adrenaline, noradrenaline, and dopamine) concentrations and mean arterial blood pressures fluctuated inappropriately during standing and sustained handgrip in 28 (82%) of the patients. The presence of headaches correlated significantly with sympathetic overactivity and instability (p less than 0.05). Resting plasma adrenaline concentrations correlated inversely with disease duration (p less than 0.05). Significant suppression of sympathetic overactivity and increases in resting lower esophageal sphincter pressures were observed in the seven patients treated with clonidine.
AD is extremely common in SSc. It is characterized by parasympathetic impairment and marked sympathetic overactivity, particularly in early disease. The potential role of AD in the pathogenesis of SSc deserves further study.
本研究旨在评估34例系统性硬化症（SSc）患者自主神经功能障碍（AD）的患病率及性质。
询问患者目前可能与AD相关的症状。进行了五项非侵入性心血管自主神经功能测试，并在静息、站立及持续握力期间对血浆儿茶酚胺进行了连续测定。对7例经测压证实有食管受累且静息血浆肾上腺素水平升高的患者给予可乐定治疗（75至375微克/天）。1个月后，重复进行静息血浆儿茶酚胺测定及食管动力研究。
自主神经测试显示所有患者均存在AD，其中33例有相关症状。与25名匹配的对照受试者相比，三项心率测试中的两项及两项血压测试的结果均有显著受损。静息血浆肾上腺素平均水平比10名匹配对照者高18倍（p<0.001）。28例（82%）患者在站立及持续握力期间血浆儿茶酚胺（肾上腺素、去甲肾上腺素和多巴胺）浓度及平均动脉血压波动异常。头痛的存在与交感神经过度活跃及不稳定显著相关（p<0.05）。静息血浆肾上腺素浓度与病程呈负相关（p<0.05）。接受可乐定治疗的7例患者交感神经过度活跃得到显著抑制，静息下食管括约肌压力增加。
AD在SSc中极为常见。其特征为副交感神经功能损害及显著的交感神经过度活跃，尤其是在疾病早期。AD在SSc发病机制中的潜在作用值得进一步研究。
