神经精神狼疮危险因素的临床分析

目的探讨神经精神狼疮(NPSLE)发病的危险因素。方法对49例NPSLE患者及107例无神经系统病变的狼疮患者进行回顾性分析,先将研究变量做单因素分析,再将有意义的变量进行多因素非条件Logistic回归分析。结果单因素分析显示起病年龄、入选年龄、病程、蝶形红斑、发热、抗Sm抗体、抗核糖体P蛋白抗体、补体C3下降(<400mg/L)与NPSLE发病显著相关(P<0.05);而正规使用激素及免疫抑制剂是具统计学意义的保护因素(P<0.05)。进一步行多因素Logistic回归分析,结果显示,发热(OR=5.865)、抗Sm抗体阳性(OR=5.156)与NPSLE发病呈正相关,有统计学意义(P<0.05);而起病年龄(OR=0.848)、病程(OR=0.959)及正规使用激素(OR=0.052)与NPSLE发病呈负相关(P<0.05)。结论起病年龄小、病程短、发热、抗Sm抗体阳性是发生NPSLE的重要因素,而正规使用糖皮质激素和免疫抑制剂是NPSLE的保护因素,医生应关注NPSLE发生的危险因素,早期给予积极治疗。     

Clinical neuropsychiatric and neuromuscular manifestations in systemic lupus erythematosus

Thirty patients with SLE were studied retrospectively and subjected to clinical neurological examination. The accumulated neurological manifestations from the beginning of the disease until the time of examination were thus collected. Twenty-five patients (83%) had experienced neuropsychiatric manifestations while 11 patients (37%) had neuromuscular manifestations. The most frequent single symptom was migraine which had occurred in 40% of the patients. This was followed by severe protracted headache in 20%, vertigo in 20%, and psychiatric problems in 17%. Carpal tunnel syndrome and muscular weakness both occurring in 23% of the patients were the most prevalent neuromuscular manifestations, followed by myositis in 10%.     

神经精神狼疮预后相关因素的临床分析

目的 探讨影响神经精神狼疮（NPSLE）患者预后相关因素。方法 对76例NPSLE患者进行回顾性分析并逐例随访至2005年12月底。先对患者的57项临床相关因素进行COX回归模型单因素分析，再将有意义的变量进行COX回归模型多因素分析．分析NPSLE预后相关因素。结果 76例NPSLE患者中共有14种神经精神症状，单因素分析显示病程、感染、横贯性脊髓炎、浆膜炎、糖尿病、中重度贫血、低蛋白血症、白球比例、蛋白尿、血小板减少、红细胞沉降率、环磷酰胺（CTX）的应用12项因素与NPSLE的预后相关（P〈0．05）。进一步行多因素分析，结果显示感染、横贯性脊髓炎、中重度贫血、血小板减少与NPSLE不良预后相关（P〈0．05），是独立的危险因素,而CTX的应用与NPSLE预后改善相关（P〈0．05），是独立的保护性因素。结论 感染、横贯性脊髓炎、血小板减少、中重度贫血是NPSLE预后的高危因素，提示预后不良；而CTX的应用是疾病预后的保护性因素，可提高NPSLE患者的生存率，应早期应用。     

Severe hypercalcemia as a presenting sign of systemic lupus erythematosus

The association of hypercalcemia and systemic lupus erythematosus is quite uncommon, with only three cases having been reported in the literature. We present a case in which this association is highlighted and demonstrate the presumed mechanism of high calcium level and the usefulness of early diagnosis and aggressive treatment.     

34例神经精神狼疮患者的临床资料分析

目的:对神经精神狼疮(NPSLE)患者的临床表现、治疗及预后进行总结。方法:对34例确诊为NPSLE住院患者的临床表现、影像学资料、实验室指标、治疗及预后等临床资料进行回顾性分析。结果:同期住院诊断SLE共818例,诊断NPSLE34例,发病率为4.16%。NPSLE首发症状最常见的是狼疮性头痛(24.2%)、脑血管病变(22.3%),发热(97.1%)及新发皮疹(61.8%)为常见的伴随症状。根据SLEDAI评分,34例患者中76.5%患者处于疾病重度活动期(评分≥15)。结论:NPSLE临床表现多样,2种及以上临床亚型并存者多见。首发症状以狼疮性头痛最常见。颅脑影像学检查在NPSLE的诊断中具有重要意义。     

Some controversies of neuropsychiatric systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is accompanied by several features that can be attributed to involvement of the central or peripheral nervous system. The etiology and pathogenesis of these manifestations are mostly unknown. To which degree these neuropsychiatric conditions can be explained on the basis of chronic illness, or as part of the disease spectrum of SLE, is also a matter of debate. Some of the controversial issues on these matters are discussed in the article.     

Antineuronal antibodies in neuropsychiatric systemic lupus erythematosus

The diagnosis of neuropsychiatric systemic lupus erythematosus (NP-SLE) is clinical and one of exclusion. Brain cross-reactive lymphocytotoxins or neuronal antibodies have been proposed as a mechanism underlying NP-SLE. We assessed the clinical relevance of neuronal cell binding antibodies using a standardized clinical definition of NP-SLE. Serum from 54 SLE patients and 77 controls were tested for binding to 3 neuroblastoma and 3 glioblastoma cell lines. Thirty-three SLE patients (61%) fulfilled clinical criteria for the diagnosis of NP-SLE; of these, 55% had serum binding activity to both neuroblastoma and glioblastoma cell lines, compared with 33% of the other SLE patients. When reactivity to neuroblastoma cell lines only was assessed, 43% of NP-SLE patient sera demonstrated binding activity, versus 14% of sera from the remaining SLE patients. Control subjects' reactivity to neuroblastoma cell lines was positive in 12% of sera. Analysis of serum reactivity using non-neuronal cell lines revealed that neuroblastoma, but not glioblastoma, cell binding was specific. NP-SLE patients with evidence of diffuse symptomatology had a higher mean titer of neuroblastoma cell line binding than those with focal symptomatology. Using a panel of substrates, one can identify a significant proportion of patients who are independently defined as having NP-SLE, who demonstrate specific serum neuronal antibodies.     

Antineuronal antibodies and neuropsychiatric systemic lupus erythematosus

Determination of antineuronal antibodies was carried out by a solid phase radioimmunoassay, in which the neuronal cells SK-N-SH were cultured as the target antigens, the positive rate in sera and CSF of neuropsychiatric SLE patients being 85% and 77.8% respectively, all with quite high level of antibodies. Although 66.7% sera of SLE patients without CNS involvement were also positive for the antibodies, yet the antibody levels of them were distinctly lower, and only 3 of them showed weak positive reactions in their CSF. It was shown that 90% of neuropsychiatric SLE patients with diffuse CNS manifestations had increased antineuronal antibodies, compared with only 20% in cases with local CNS involvements. The antibody levels both in sera and CSF decreased remarkably following the patients' recovery from the neuropsychiatric attack. It is concluded that the antineuronal antibodies might play a role in the pathogenesis of neuropsychiatric lupus and the determination of them in CSF might be useful in the diagnosis and differential diagnosis of neuropsychiatric lupus.     

Magnetic resonance imaging in systemic lupus erythematosus patients without a history of neuropsychiatric lupus erythematosus

Objective. To determine the prevalence of magnetic resonance imaging (MRI) lesions in systemic lupus erythematosus (SLE) patients without a history of neuropsychiatric symptoms and to correlate any MRI abnormalities with the patient's other disease manifestations or treatment. Methods. Prospective study of 32 consecutive patients with SLE without a history of neuropsychiatric symptoms, from inpatient and outpatient rheumatology services, who underwent MRI scan during a 3-year period. Results. Five patients had MRI abnormalities consisting of white matter lesions or periventricular hyperintensities; this is similar to the prevalence of these abnormalities in the general population. Conclusion. The prevalence of silent brain MRI abnormalities is not increased in SLE patients who do not have a history of neuropsychiatric manifestations.     

DNA as a marker of cell death in systemic lupus erythematosus

DNA circulates in the blood in systemic lupus erythematosus, among other conditions, and plays a role in immunopathogenesis in the form of immune complexes. As shown in experiments in mice, blood DNA levels rise following treatments to induce apoptosis and the administration of cells made apoptotic or necrotic in vitro. In mice lacking macrophage function, however, blood levels do not rise following administration of dead cells. These results indicate that circulating DNA may be a marker of cell death, although its levels likely reflect a complex process involving the interactions of macrophages with dead and dying cells.     

Mimickers of neuropsychiatric manifestations in systemic lupus erythematosus

Systemic lupus erythematosus (SLE), presenting with new onset or worsening neuropsychiatric (NP) symptoms, is a challenge in clinical practice. Mimickers such as infections, drug-induced side effects, metabolic abnormalities, malignancies, and alcohol-related disorders have to be excluded, before attributing the manifestations to disease activity. Proper diagnosis is essential to guide adequate management and reduce morbidity and mortality. In this review article, we will highlight clinical, laboratorial, and neuroradiological features that are helpful to assist in the differential diagnosis.     

Antineurofilament antibody evaluation in neuropsychiatric systemic lupus erythematosus

We used Western blot analysis to examine the occurrence and titer of antibody to cytoskeletal neurofilament protein antigens in patients with neuropsychiatric manifestations of systemic lupus erythematosus (SLE) and in controls. Twenty-two patients with neuropsychiatric SLE (NPSLE) had an increased incidence of antineurofilament antibody (ANFA) compared with 34 patients with SLE without neuropsychiatric symptoms, 78 patients with other disease processes, and 22 healthy controls. ANFA were found to be directed against the 205,000- and 160,000-dalton proteins of the neurofilament triplet. Patients with a diffuse NPSLE clinical presentation had the greatest frequency of serum ANFA (7 of 12, 58%) compared with all other groups examined. Magnetic resonance imaging and serum anticardiolipin antibody testing were also performed in selected patients with NPSLE. Patients with a focal clinical presentation of NPSLE, positive magnetic resonance imaging findings, and negative serum ANFA had significantly elevated levels of anticardiolipin antibody.