Min Liliane, Zwerling Jessica, Ocava Lenore Concepcion, Chen I-Hweii Amy, Putterman Chaim
Division of Rheumatology, Department of Medicine, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
Semin Arthritis Rheum. 2006 Jun;35(6):388-95. doi: 10.1016/j.semarthrit.2006.01.003.
To describe a case of reversible posterior leukoencephalopathy (RPLS) involving a patient with systemic lupus erythematosus (SLE) and to review the medical literature to define the epidemiological, clinical, radiological, and therapeutic aspects of this syndrome in various connective tissue diseases.
Report of 1 case and review of the English literature using Medline search from 1967 to 2005.
Including our reported case, RPLS has been identified in 13 patients with connective tissue disease. In separate case reports, 9 SLE patients, 2 Wegener's granulomatosis (WG) patients, and 1 patient with SLE and systemic sclerosis presented with RPLS. Associated risk factors included malignant hypertension, acute renal failure, and recent treatment with cyclophosphamide, cyclosporine, or methylprednisolone. Patients were treated with blood pressure control, hemodialysis, or withdrawal of the offending drug. In our patient, plasmapheresis and high-dose methylprednisolone resulted in a full recovery. In most cases, complete resolution of neurological symptoms occur within 2 weeks of presentation, along with improvement or resolution of imaging abnormalities.
RPLS is a clinicoradiological entity, associated with reversible white matter edema involving most commonly the posterior central nervous system circulation. Seizures and altered mental status in patients with SLE or WG can pose difficult diagnostic and therapeutic challenges. The differential diagnosis is broad and includes infection, uremia, hypertension, infarction, thrombosis, demyelinating disorders, and vasculitis. Accurate diagnosis of RPLS and its differentiation from other, more common causes of the central nervous system is essential to ensure the best possible outcome in this rare but life-threatening neurological disorder.
描述一例累及系统性红斑狼疮（SLE）患者的可逆性后部白质脑病（RPLS）病例，并回顾医学文献以明确该综合征在各种结缔组织疾病中的流行病学、临床、放射学及治疗方面的情况。
报告1例病例，并使用1967年至2005年的Medline搜索对英文文献进行回顾。
包括我们报告的病例在内，已在13例结缔组织病患者中识别出RPLS。在单独的病例报告中，9例SLE患者、2例韦格纳肉芽肿（WG）患者以及1例患有SLE和系统性硬化症的患者出现了RPLS。相关危险因素包括恶性高血压、急性肾衰竭以及近期使用环磷酰胺、环孢素或甲泼尼龙治疗。患者接受了血压控制、血液透析或停用致病药物的治疗。在我们的患者中，血浆置换和大剂量甲泼尼龙治疗后完全康复。在大多数情况下，神经症状在出现后2周内完全缓解，同时影像学异常也有所改善或消失。
RPLS是一种临床放射学实体，与可逆性白质水肿相关，最常累及中枢神经系统后循环。SLE或WG患者出现癫痫发作和精神状态改变可能带来诊断和治疗方面的难题。鉴别诊断范围广泛，包括感染、尿毒症、高血压、梗死、血栓形成、脱髓鞘疾病和血管炎。准确诊断RPLS并将其与其他更常见的中枢神经系统病因相鉴别，对于确保这种罕见但危及生命的神经系统疾病获得最佳治疗效果至关重要。
