Mevorach D, Raz E, Steiner I
Department of Internal Medicine, Hadassah University Hospital, Mount Scopus, Jerusalem, Israel.
Lupus. 1994 Apr;3(2):117-21. doi: 10.1177/096120339400300211.
The diagnosis of systemic lupus erythematosus (SLE) in a patient who presents with isolated central nervous system (CNS) abnormalities, is a difficult clinical challenge. The pathogenesis of such CNS involvement in SLE is unknown. Twelve patients with active SLE were examined for serum and cerebrospinal fluid (CSF) autoantibodies and compared with 21 patients with headache. Four of six patients with SLE and active CNS presentation had CSF autoantibodies while none of the other SLE patients and the controls had autoantibodies. We describe these four patients in whom immunological work-up revealed CSF antinuclear, anti-DNA, anti-SSA/Ro, anti-SSB/La and antineuronal autoantibodies. A newly devised antibody activity index provided means to demonstrate that the CSF autoantibodies in these patients were produced intrathecally. Beyond the importance of our finding of the potential role of autoantibodies in the pathogenesis of CNS damage in SLE, we propose that CSF analysis for autoantibodies should become an essential part of the diagnostic work-up in autoimmune diseases with CNS involvement.
对于仅表现为中枢神经系统（CNS）异常的患者，诊断系统性红斑狼疮（SLE）是一项艰巨的临床挑战。SLE患者中枢神经系统受累的发病机制尚不清楚。对12例活动性SLE患者进行了血清和脑脊液（CSF）自身抗体检测，并与21例头痛患者进行了比较。6例有活动性中枢神经系统表现的SLE患者中有4例脑脊液存在自身抗体，而其他SLE患者及对照组均无自身抗体。我们描述了这4例患者，其免疫检查显示脑脊液中有抗核抗体、抗DNA抗体、抗SSA/Ro抗体、抗SSB/La抗体和抗神经元自身抗体。一种新设计的抗体活性指数提供了方法来证明这些患者脑脊液中的自身抗体是鞘内产生的。除了我们发现自身抗体在SLE中枢神经系统损伤发病机制中的潜在作用具有重要意义外，我们建议对自身抗体进行脑脊液分析应成为中枢神经系统受累的自身免疫性疾病诊断检查的重要组成部分。
