Jodo S, Sagawa A, Ogura N, Atsumi T, Amasaki Y, Nakabayashi T, Watanabe I, Mukai M, Fujisaku A, Nakagawa S
Second Department of Internal Medicine, Hokkaido University, Sapporo-city.
Ryumachi. 1992 Feb;32(1):58-65.
A 43-year-old woman who had been diagnosed as primary Sjögren's syndrome since 1986 developed severe constipation, urinary retention, dizziness at standing and polyarthralgia in February, 1990. Laboratory tests revealed proteinuria, hypocomplementemia and high titer of anti-DNA antibody. Diagnosis of SLE was made and she was admitted to our hospital on April 2, 1990. Physical examination on admission showed that she also had asymmetric pupils, impairment of sweating, orthostatic hypotension, neurogenic bladder, gastro-intestinal dysmotility and the diminution of R-R interval variability during deep breathing on the electrocardiogram. These findings suggested that she had pan-dysautonomia but there were no signs of motor and sensory disturbance. Because other diseases such as diabetes mellitus and amyloidosis which induced dysautonomia could be ruled out, her pan-dysautonomia seemed to be due to SLE. After the treatment with steroid pulse therapy, most of her dysautonomia improved rapidly. However, some of the disturbance had persisted for a long time. Pan-dysautonomia has been rarely reported as a complication of SLE, and high dose of steroid therapy at the early stage should be considered.
一名自1986年起被诊断为原发性干燥综合征的43岁女性，于1990年2月出现严重便秘、尿潴留、站立时头晕和多关节痛。实验室检查显示蛋白尿、低补体血症和高滴度抗DNA抗体。诊断为系统性红斑狼疮（SLE），并于1990年4月2日入住我院。入院时体格检查发现她还存在瞳孔不对称、出汗障碍、体位性低血压、神经源性膀胱、胃肠动力障碍以及心电图上深呼吸时R-R间期变异性减小。这些发现提示她患有全自主神经功能不全，但无运动和感觉障碍的体征。由于可以排除其他导致自主神经功能不全的疾病，如糖尿病和淀粉样变性，她的全自主神经功能不全似乎是由SLE引起的。经过类固醇冲击治疗后，她的大部分自主神经功能不全迅速改善。然而，一些功能障碍持续了很长时间。全自主神经功能不全作为SLE的并发症很少见，早期应考虑大剂量类固醇治疗。
