Vadacca M, Buzzulini F, Rigon A, Coppolino G, Palma Modoni A, Massa R, Afeltra A
Dipartimento di Medicina Clinica, Immunologia, Reumatologia, Università Campus Bio-Medico, Roma, Italia.
Reumatismo. 2006 Jul-Sep;58(3):177-86. doi: 10.4081/reumatismo.2006.177.
Neuropsychiatric involvement in patients with Systemic Lupus Erythematosus (SLE), first mentioned by Kaposi more than 100 years ago, still remains one of the main challenge facing rheumatologist and other physicians. The diagnosis of neuropsychiatric SLE (NPSLE) is complex not only because of the considerable prevalence variation (14-80%) but also because of the wide spectrum of NP manifestations. They vary from overt neurologic alterations (seizure, psychosis), to subtle abnormalities (neurocognitive dysfunctions). Different NP manifestations result from a variety of mechanisms including antibodies, vasculitis, thrombosis, hemorrhages and cytokine-mediated damages. Of note, despite the dramatic clinical manifestations, too often changes at the morphological neuroimaging techniques are minimal and non specific. There is no one diagnostic tool specific for NPSLE and diagnosis must be based on the combinated use of immunoserological tests, functional and anatomical neuroimaging and standardized specific criteria. Symptomatic, immunosuppressive and anticoagulant therapies are the main strategies available in the management of these patients. Therapy for CNS lupus should be adjusted according to the needs of the individual patients. The coming years promise to be an important time for the development of new neuroimaging techniques and for the study of disease mechanism. An early and objective identification of brain involvement will allow for appropriate treatment to avoid severe complications.
100多年前卡波西首次提到系统性红斑狼疮(SLE)患者的神经精神受累,这仍然是风湿病学家和其他医生面临的主要挑战之一。神经精神性狼疮(NPSLE)的诊断很复杂,不仅因为其患病率差异很大(14%-80%),还因为NP表现的范围很广。其表现从明显的神经改变(癫痫发作、精神病)到细微异常(神经认知功能障碍)不等。不同的NP表现由多种机制引起,包括抗体、血管炎、血栓形成、出血和细胞因子介导的损伤。值得注意的是,尽管临床表现显著,但形态学神经成像技术的改变往往很小且不具有特异性。没有一种特定的诊断工具可用于NPSLE,诊断必须基于免疫血清学检测、功能和解剖神经成像以及标准化的特定标准的联合使用。对症治疗、免疫抑制治疗和抗凝治疗是这些患者管理中的主要可用策略。中枢神经系统狼疮的治疗应根据个体患者的需求进行调整。未来几年有望成为新神经成像技术发展和疾病机制研究的重要时期。早期客观地识别脑部受累将有助于进行适当治疗以避免严重并发症。