Winfield J B, Shaw M, Silverman L M, Eisenberg R A, Wilson H A, Koffler D
Am J Med. 1983 May;74(5):837-44. doi: 10.1016/0002-9343(83)91075-6.
Paired serum and cerebrospinal fluid specimens from 19 patients with SLE and central nervous system dysfunction were studied with respect to cerebrospinal fluid IgG index (a measure of intrathecal IgG synthesis), isoelectric focusing using immunoperoxidase staining techniques to detect oligoclonal IgG, and determination of the cerebrospinal fluid/serum albumin quotient (Q albumin) as a measure of blood-brain barrier integrity. Twenty-five patients without neurologic disease and 70 patients with a variety of non-SLE neurologic disorders were also studied for comparison. Of most interest was the observation that 42 percent of the patients with SLE had cerebrospinal fluid oligoclonal IgG, usually in association with elevation of the cerebrospinal fluid IgG index. In addition, two of the cerebrospinal fluid specimens that exhibited oligoclonal IgG also had increased titers of alpha-interferon. Q albumin was normal (under 9.0) in 12 of 13 patients with SLE, who had seizure, psychosis, or cranial neuropathy as principal central nervous system manifestations (mean +/- SD = 5.3 +/- 2.4), but was significantly elevated (mean +/- SD = 27.4 +/- 18.8, p less than 0.001) in five of six patients with diffuse, major central nervous system injury, for example, encephalopathy with coma, transverse myelopathy, paraparesis. Blood-brain barrier impairment was not correlated either with presence of circulating immune complexes or with other clinical or serologic evidence for extra-central nervous system disease activity. Taken together, the data suggest that, within the limitations of the techniques used, impairment of the blood-brain barrier in SLE may be secondary to the central nervous system lesion, rather than a result of systemic immune complex injury. In addition, substantial evidence is provided for an ongoing humoral immune response within the central nervous system in this disorder, which, in certain patients, may be associated with the production of intrathecal alpha-interferon.
对19例患有系统性红斑狼疮（SLE）且伴有中枢神经系统功能障碍的患者，采集其配对的血清和脑脊液标本，研究脑脊液IgG指数（衡量鞘内IgG合成的指标）、采用免疫过氧化物酶染色技术进行等电聚焦以检测寡克隆IgG，以及测定脑脊液/血清白蛋白商（Q白蛋白）以评估血脑屏障的完整性。还对25例无神经系统疾病的患者以及70例患有各种非SLE神经系统疾病的患者进行了研究以作比较。最令人感兴趣的观察结果是，42%的SLE患者脑脊液中有寡克隆IgG，通常与脑脊液IgG指数升高有关。此外，两份显示有寡克隆IgG的脑脊液标本中α干扰素的滴度也有所升高。13例以癫痫、精神病或颅神经病变为主要中枢神经系统表现的SLE患者中，12例的Q白蛋白正常（低于9.0）（均值±标准差 = 5.3±2.4），但在6例患有弥漫性严重中枢神经系统损伤（如伴有昏迷的脑病、横贯性脊髓炎、轻截瘫）的患者中，有5例的Q白蛋白显著升高（均值±标准差 = 27.4±18.8，p<0.001）。血脑屏障损害与循环免疫复合物的存在或中枢神经系统外疾病活动的其他临床或血清学证据均无关联。综合来看这些数据表明，在所使用技术的局限性范围内，SLE中的血脑屏障损害可能继发于中枢神经系统病变，而非全身性免疫复合物损伤的结果。此外，有大量证据表明该疾病中枢神经系统内存在持续的体液免疫反应，在某些患者中，这可能与鞘内α干扰素的产生有关。
