West S G, Emlen W, Wener M H, Kotzin B L
Rheumatology Service, Fitzsimons Army Medical Center, Aurora, Colorado 80045-5001, USA.
Am J Med. 1995 Aug;99(2):153-63. doi: 10.1016/s0002-9343(99)80135-1.
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).
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.
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.
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.
评估哪些血清学、脑脊液（CSF）和神经影像学检查单独或联合使用对神经精神性狼疮（NPLE）的诊断最有帮助。
对1982年1月至1991年12月因神经精神疾病住院的系统性红斑狼疮（SLE）患者进行前瞻性研究。作为本研究一部分评估的特殊检查包括血清抗核抗体、补体水平、血清和脑脊液抗神经元抗体、脑脊液特殊蛋白研究（免疫球蛋白G [IgG]指数和寡克隆带）、血清抗核糖体-P抗体、血清抗磷脂抗体以及头颅磁共振成像（MRI）。确定单项检查及检查组合的诊断敏感性、特异性和阳性预测值（PPV）。
52例NPLE患者按神经精神表现分类（32例弥漫性、10例局灶性和10例复杂性表现），并与14例SLE对照患者进行比较。每例有弥漫性或复杂性表现的NPLE患者脑脊液IgG指数/寡克隆带异常、脑脊液抗神经元抗体升高和/或血清抗核糖体-P抗体升高，该检查组合的敏感性为100%，特异性为86%，PPV为95%。10例局灶性表现患者中有9例以及所有复杂性疾病患者有血管炎/网状青斑、抗磷脂抗体和/或头颅MRI有多处病变的证据，该组检查的敏感性为95%，特异性为86%，PPV为90%。这些检查组合正确诊断了所有9例根据后续临床病程最初诊断被证明错误的SLE患者。随着治疗成功，异常检查结果常恢复正常或改善。
脑脊液抗体的特异性检查在弥漫性NPLE诊断中最有帮助，提示自身抗体在这种NPLE表现的发病机制中起作用。在那些主要表现为精神疾病的弥漫性NPLE患者中，血清抗核糖体-P抗体似乎有帮助。相比之下，局灶性NPLE似乎主要继发于血管闭塞，皮肤血管炎/网状青斑、抗磷脂抗体和/或头颅MRI异常在诊断中最有帮助。复杂性表现患者表现出弥漫性和局灶性NPLE的特征性异常。异常检查可连续监测，且似乎与治疗的临床反应相关。
