Neuwelt C M, Lacks S, Kaye B R, Ellman J B, Borenstein D G
Department of Medicine, Highland General Hospital, Oakland, California.
Am J Med. 1995 Jan;98(1):32-41. doi: 10.1016/S0002-9343(99)80078-3.
We assessed the outcome of 31 patients with severe neuropsychiatric (NP) systemic lupus erythematosus (NPSLE) treated with intravenous cyclophosphamide (IV-CYC), and identified clinical predictors of response to therapy.
The authors performed a retrospective chart review and classified patients by NP manifestation and response to therapy as measured by serial anatomic imaging and neurodiagnostic studies coupled with clinical assessment of improvement.
Neuropsychiatric manifestations occurred with the following frequencies: organic brain syndromes (OBS) 55%, stroke syndromes 35%, peripheral or mononeuropathy 32%, seizures 29%, psychiatric symptoms 26%, transverse myelitis 16%, cranial neuropathies 13%, other 16%. Most patients had multiple NP manifestations, with a median of two. Ninety percent of patients had failed therapy with corticosteroids with or without cytotoxic drugs prior to treatment with IV-CYC. Eight patients received synchronous plasmapheresis along with IV-CYC. After treatment with IV-CYC, NP deficits substantially improved in 61% (group I), stabilized in 29% (group S), and progressively deteriorated in 10% (group P). Patients in group I had significantly fewer NP manifestations than combined group S+P, two versus four, and a lower frequency of OBS, 37% versus 83%.
Intravenous cyclophosphamide appears to be an effective treatment for some patients with severe NPSLE refractory to other forms of therapy. Higher number of NP manifestations and presence of OBS may predict poor outcome and identify a group of patients for whom early aggressive therapy may be indicated.
我们评估了31例接受静脉注射环磷酰胺（IV-CYC）治疗的重症神经精神性（NP）系统性红斑狼疮（NPSLE）患者的治疗结果，并确定了治疗反应的临床预测因素。
作者进行了一项回顾性病历审查，根据NP表现以及通过系列解剖成像和神经诊断研究并结合改善情况的临床评估来衡量的治疗反应对患者进行分类。
神经精神性表现出现的频率如下：器质性脑综合征（OBS）55%，中风综合征35%，周围或单神经病32%，癫痫发作29%，精神症状26%，横贯性脊髓炎16%，颅神经病变13%，其他16%。大多数患者有多种NP表现，中位数为两种。90%的患者在接受IV-CYC治疗前使用或未使用细胞毒性药物的皮质类固醇治疗均失败。8例患者在接受IV-CYC治疗的同时进行了同步血浆置换。接受IV-CYC治疗后，61%的患者（I组）NP缺陷显著改善，29%的患者（S组）病情稳定，10%的患者（P组）病情逐渐恶化。I组患者的NP表现明显少于S+P联合组，分别为两种和四种，且OBS的发生率较低，分别为37%和83%。
静脉注射环磷酰胺似乎是治疗某些对其他治疗形式难治的重症NPSLE患者的有效方法。NP表现数量较多和存在OBS可能预示预后不良，并确定一组可能需要早期积极治疗的患者。
