Hirohata S, Miyamoto T
J Rheumatol. 1986 Aug;13(4):715-21.
The concentration of immunoglobulins in paired serum and cerebrospinal fluid (CSF) specimens from 15 patients with systemic lupus erythematosus (SLE) and central nervous system (CNS) involvement (CNS-SLE) were determined based on their light chain properties. Eight patients with SLE without CNS involvement and 20 patients with noninflammatory neurological diseases were also studied for comparison. We found that both of the CSF Ig-k (immunoglobulin kappa) and Ig-1 (immunoglobulin lambda) indices were significantly elevated, indicating the increased intrathecal immunoglobulin synthesis of both kappa and lambda types in patients with CNS-SLE. Moreover, the CSF Ig-k and Ig-1 indices correlated well with each other, and both indices were found to decrease remarkably when the CNS manifestations subsided after successful treatment. In 4 of 15 patients, intrathecal synthesis of free light chains in addition to Ig bound light chains seemed to be increased. These observations provide substantial evidence for polyclonal B lymphocyte activation within the CNS.
基于轻链特性，测定了15例患有系统性红斑狼疮（SLE）且累及中枢神经系统（CNS）（CNS-SLE）患者配对的血清和脑脊液（CSF）标本中免疫球蛋白的浓度。还研究了8例无CNS受累的SLE患者和20例非炎性神经系统疾病患者作为对照。我们发现，CSF的Ig-k（免疫球蛋白κ）和Ig-λ（免疫球蛋白λ）指数均显著升高，表明CNS-SLE患者鞘内κ型和λ型免疫球蛋白合成均增加。此外，CSF的Ig-k和Ig-λ指数相互之间具有良好的相关性，并且在成功治疗后CNS表现消退时，发现这两个指数均显著下降。在15例患者中的4例中，除了与Ig结合的轻链外，游离轻链的鞘内合成似乎也增加。这些观察结果为CNS内多克隆B淋巴细胞激活提供了大量证据。
