NEUROPSYCHIATRIC LUPUS-ERYTHEMATOSUS - A 10-YEAR PROSPECTIVE-STUDY ON THE VALUE OF DIAGNOSTIC-TESTS
NEUROPSYCHIATRIC LUPUS-ERYTHEMATOSUS - A 10-YEAR PROSPECTIVE-STUDY ON THE VALUE OF DIAGNOSTIC-TESTS
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DOI：
10.1016/s0002-9343(99)80135-1
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发表时间：
1995-08-01
影响因子：
5.9
通讯作者：
KOTZIN, BL
中科院分区：
文献类型：
--
作者：
WEST, SG;EMLEN, W;KOTZIN, BL
目得：为了评估血清学，脑脊液（CSF），神经放射学测试单独或组合是最有用的诊断neuropsychiatric lupus erythematosus（NPLE）.Patients AND Methods：前瞻性研究系统性红斑狼疮（SLE）患者住院与神经精神疾病1982年1月至1991年12月。作为本研究一部分进行评价的特殊检查包括血清抗核抗体、补体水平、血清和CSF抗神经元抗体、CSF特殊蛋白研究（免疫球蛋白G [IgG]指数和寡克隆带）、血清抗核糖体-P抗体、血清抗磷脂抗体和头颅磁共振成像（MRI）。诊断的敏感性，特异性和阳性预测值（PPV）确定为单一的测试和组合tests.Results：五十二NPLE患者进行了分类的神经精神的介绍（32弥漫性，10局灶性，10复杂的介绍），并与14个SLE对照患者。每个弥漫性或复杂表现的NPLE患者都有异常的CSF IgG指数/寡克隆带，升高的CSF抗神经元抗体和/或血清抗核糖体-P抗体，对于这种测试组合，灵敏度为100%，特异性为86%，PPV为95%。10例局灶性表现的患者中有9例有血管炎/网状青斑、抗磷脂抗体和/或头颅MRI多发性病变的证据，该系列试验的敏感性为95%，特异性为86%，PPV为90%。这些测试的组合正确地诊断了所有9名SLE患者，这些患者的初步诊断被证明是不正确的，基于随后的临床过程。异常的测试结果经常正常化或改善成功therapy.CONCLUSIONS：特异性测试CSF抗体是最有用的诊断弥漫性NPLE，牵连自身抗体在发病机制，这NPLE介绍。在弥漫性NPLE患者中，主要表现为精神疾病，血清抗核糖体-P抗体似乎是有帮助的。相比之下，局灶性NPLE似乎主要继发于血管闭塞，并且皮肤血管炎/网状青斑、抗磷脂抗体和/或异常头颅MRI的存在对诊断最有帮助。复杂表现的患者表现出弥漫性和局灶性NPLE的异常特征。异常检测可以连续进行，并且似乎与对治疗的临床反应相关。
PURPOSE: To evaluate which serologic, cerebrospinal fluid (CSF), and neuroradiographic tests alone or in combination are most useful in the diagnosis of neuropsychiatric lupus erythematosus (NPLE).PATIENTS AND METHODS: Prospective study of patients with systemic lupus erythematosus (SLE) hospitalized with neuropsychiatric disease between January 1982 and December 1991. Special tests evaluated as part of this study included serum antinuclear antibodies, complement levels, serum and CSF antineuronal antibodies, CSF special protein studies (immunoglobulin G [IgG] index and oligoclonal bands), serum antiribosomal-P antibodies, serum antiphospholipid antibodies, and cranial magnetic resonance imaging (MRI). Diagnostic sensitivity, specificity, and positive predictive value (PPV) were determined for single tests and combinations of tests.RESULTS: Fifty-two NPLE patients were categorized by neuropsychiatric presentation (32 diffuse, 10 focal, and 10 complex presentations) and compared to 14 SLE control patients. Each NPLE patient with a diffuse or complex presentation had abnormal CSF IgG index/oligoclonal bands, elevated CSF antineuronal antibodies, and/or serum antiribosomal-P antibodies, yielding a sensitivity of 100%, specificity of 86%, and PPV of 95% for this combination of tests. Nine of 10 patients with focal presentations and all with complex disease had evidence of vasculitis/livedo reticularis, antiphospholipid antibodies, and/or a cranial MRI with multiple lesions, giving a sensitivity of 95%, specificity of 86%, and a PPV of 90% for this battery of tests. These combinations of tests correctly diagnosed all nine SLE patients whose initial diagnosis proved to be incorrect based on subsequent clinical course. Abnormal test results frequently normalized or improved with successful therapy.CONCLUSIONS: Specific tests for CSF antibodies are most useful diagnostically in diffuse NPLE, implicating autoantibodies in the pathogenesis of this NPLE presentation. In those patients with diffuse NPLE who present with primarily psychiatric disease, serum antiribosomal-P antibodies appear to be helpful. In contrast, focal NPLE appears to be mostly secondary to vascular occlusion, and the presence of dermal vasculitis/livedo reticularis, antiphospholipid antibodies, and/or an abnormal cranial MRI are most helpful diagnostically. Patients with complex presentations demonstrate abnormalities characteristic of both diffuse and focal NPLE. Abnormal tests can be followed serially and appear to correlate with clinical responses to therapy.