Tincani A, Brey R, Balestrieri G, Vitali C, Doria A, Galeazzi M, Meroni P L, Migliorini P, Neri R, Tavoni A, Bombardieri S
Clinical Immunology Unit, Ospedale Civile di Brescia, Italy.
Clin Exp Rheumatol. 1996 Nov-Dec;14 Suppl 16:S23-9.
To determine the diagnostic and therapeutic approach used in clinical practice for the management of systemic lupus erythematosus (SLE) patients with primary SLE-mediated neuropsychiatric (NP) manifestations.
A questionnaire was drawn up to assess how clinicians manage various clinical manifestations of SLE. A portion of this questionnaire was designed to assess how clinicians diagnose and treat primary NP-SLE. Most of the questions in the NP-SLE section consisted of lists of different clinical manifestations and laboratory or radiological studies that participants were asked to rate on a scale of importance [from 1 (extremely important) to 5 (not important)] to the diagnosis of primary NP-SLE. The questionnaire also assessed how different NP manifestations are treated in clinical practice. The relative importance of each clinical manifestation was determined through its mean score, and the agreement among participants on each issue was determined using the coefficient of variation (CV). Fifty-nine lupus centers participated in the NP-SLE portion of the survey.
The clinical manifestations which were considered to be of extreme or major importance for the diagnosis of primary NP-SLE were seizures, psychosis, transverse myelitis, stroke, transient ischemic attack (TIA) and aseptic meningitis. Among the radiological and laboratory studies, only brain magnetic resonance imaging (MRI) and antiphospholipid antibodies (aPL) achieved "extremely important" mean scores (between 1 and 2). aPL testing was used routinely in the majority of patients (mean 96.8%; CV = 0.1), while brain MRI was used less frequently (mean 56.5%; CV = 0.61). Only brain MRI and cerebral angiography were considered to be helpful in differentiating cerebral vasculopathy from multi-infarct (mean score = 1.6 and 1.9, respectively), whereas a prompt response to treatment with increased doses of steroids was considered helpful in differentiating SLE-related psychosis from steroid-induced psychosis (mean score = 1.58). The results of aPL testing, coagulation tests for the lupus anticoagulant, an brain MRI were considered to be of extreme or major importance in decisions involving treatment with anticoagulant or anti-platelet therapy. Symptomatic therapies, such as heparin, or anti-convulsant, anti-platelet, oral anticoagulant, and antipsychotic therapy were the most widely used. Corticosteroids were the most frequently used immunosuppressive therapy. The administration of other immunosuppressive agents as specific treatment for NP-SLE was uncommon.
Our survey found that in clinical practice, the NP manifestations currently considered to be diagnostic of primary SLE-mediated CNS involvement are not limited to those included in the American Rheumatism Association (ARA) criteria, e.g. seizures and psychosis. Antiphospholipid antibodies appeared to be the laboratory parameter most frequently relied upon in the diagnosis of NP-SLE, and in decisions regarding treatment. Apart from that, only brain MRI and, in selected cases, cerebral angiography seemed to be of real help in diagnosis. The lack of consensus regarding the treatment of primary NP-SLE manifestations most probably reflects both the complex nature of neurological illness in SLE patients and the lack of clear diagnostic criteria.
确定临床实践中用于管理患有原发性系统性红斑狼疮（SLE）介导的神经精神（NP）表现的SLE患者的诊断和治疗方法。
编制了一份问卷，以评估临床医生如何管理SLE的各种临床表现。该问卷的一部分旨在评估临床医生如何诊断和治疗原发性NP-SLE。NP-SLE部分的大多数问题由不同临床表现以及实验室或影像学检查的列表组成，要求参与者根据对原发性NP-SLE诊断的重要性程度[从1（极其重要）到5（不重要）]进行评分。问卷还评估了在临床实践中如何治疗不同的NP表现。通过平均得分确定每种临床表现的相对重要性，并使用变异系数（CV）确定参与者在每个问题上的一致性。59个狼疮中心参与了该调查的NP-SLE部分。
对于原发性NP-SLE的诊断，被认为极其重要或非常重要的临床表现为癫痫发作、精神病、横贯性脊髓炎、中风、短暂性脑缺血发作（TIA）和无菌性脑膜炎。在影像学和实验室检查中，只有脑磁共振成像（MRI）和抗磷脂抗体（aPL）的平均得分达到“极其重要”（介于1和2之间）。大多数患者常规进行aPL检测（平均96.8%；CV = 0.1），而脑MRI的使用频率较低（平均56.5%；CV = 0.61）。只有脑MRI和脑血管造影被认为有助于区分脑血管病变与多发性梗死（平均得分分别为1.6和1.9），而对增加剂量类固醇治疗的快速反应被认为有助于区分SLE相关精神病与类固醇诱导的精神病（平均得分 = 1.58）。aPL检测结果、狼疮抗凝物的凝血试验以及脑MRI在涉及抗凝或抗血小板治疗的决策中被认为极其重要或非常重要。对症治疗，如肝素、抗惊厥药、抗血小板药、口服抗凝药和抗精神病治疗是使用最广泛的。皮质类固醇是最常用的免疫抑制治疗。使用其他免疫抑制剂作为NP-SLE的特异性治疗并不常见。
我们的调查发现，在临床实践中，目前被认为可诊断原发性SLE介导的中枢神经系统受累的NP表现并不局限于美国风湿病协会（ARA）标准中所包含的那些，例如癫痫发作和精神病。抗磷脂抗体似乎是NP-SLE诊断以及治疗决策中最常依赖的实验室参数。除此之外，只有脑MRI以及在某些情况下的脑血管造影似乎对诊断有实际帮助。对于原发性NP-SLE表现的治疗缺乏共识很可能既反映了SLE患者神经系统疾病的复杂性，也反映了缺乏明确的诊断标准。
