Lioger Bertrand, Diot Elisabeth, Ebbo Michael, Schleinitz Nicolas, Aaron Laurent, Michot Jean-Marie, Lambotte Olivier, Dhote Robin, De Boysson Hubert, Ponce Elodie, Maillot François
CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
CHRU de Tours, Service de Médecine Interne, Tours, France.
Clin Exp Rheumatol. 2016 May-Jun;34(3 Suppl 97):S7-11. Epub 2015 Sep 28.
Our objective was to describe the characteristics of posterior reversible encephalopathy syndrome (PRES) associated with systemic vasculitis.
A standardised questionnaire was used for a nationwide retrospective multicentre study in 2013 to collect clinical, radiological and outcome data about PRES associated with systemic vasculitis.
We included six patients (all women; mean age 22.6±19.8 years (20-62)): two with polyarteritis nodosa and one case of each granulomatosis with polyangiitis, cryoglobulinaemic vasculitis, hypocomplementemic urticarial vasculitis, and Takayasu arteritis. PRES was the first manifestation of systemic vasculitis in three patients. Arterial hypertension was suspected to be the cause of PRES in five patients. Several other plausible causes including drugs, renal failure, and pneumonia were found in three patients. Clinical findings included headache, seizure, blurred or loss of vision, confusion, and altered cognition. Radiological study showed oedema in the occipital region in all patients, with a reversible state in MRIs performed one week to one month after the onset of PRES. Therapies used included antihypertensive therapy (n=5), immunosuppressive therapy (corticosteroids (n=5), cyclophosphamide (n=4), azathioprine (n=1), methotrexate (n=1), plasma exchange (n=1)), antibiotics (n=1), anticonvulsant therapy (n=2)), and analgesics. No relapse of PRES was reported during the follow-up period (mean: 47.5 ±29.9 months, 13-98); one patient continued to complain of vision loss.
Our study indicates that PRES is a rare condition associated with systemic vasculitis; which may be present at the onset vasculitis symptoms. Antihypertensive drugs should be prescribed if blood pressure is elevated. The impact of immunosuppressive therapy remains unclear.
我们的目的是描述与系统性血管炎相关的后部可逆性脑病综合征（PRES）的特征。
2013年在全国范围内进行了一项标准化问卷调查的回顾性多中心研究，以收集与系统性血管炎相关的PRES的临床、放射学和预后数据。
我们纳入了6例患者（均为女性；平均年龄22.6±19.8岁（20 - 62岁））：2例结节性多动脉炎，1例肉芽肿性多血管炎、冷球蛋白血症性血管炎、低补体血症性荨麻疹性血管炎和大动脉炎各1例。PRES是3例患者系统性血管炎的首发表现。5例患者疑似动脉高血压是PRES的病因。3例患者还发现了其他一些可能的病因，包括药物、肾衰竭和肺炎。临床表现包括头痛、癫痫发作、视力模糊或丧失、意识模糊和认知改变。放射学研究显示所有患者枕叶区域有水肿，在PRES发病后1周内至1个月进行的MRI检查显示为可逆状态。使用的治疗方法包括抗高血压治疗（n = 5）、免疫抑制治疗（皮质类固醇（n = 5）、环磷酰胺（n = 4）、硫唑嘌呤（n = 1）、甲氨蝶呤（n = 1）、血浆置换（n = 1））、抗生素（n = 1）、抗惊厥治疗（n = 2）和镇痛药。随访期间（平均：47.5±29.9个月，13 - 98个月）未报告PRES复发；1例患者仍抱怨视力丧失。
我们的研究表明，PRES是一种与系统性血管炎相关的罕见疾病；可能在血管炎症状出现时就存在。如果血压升高，应开具抗高血压药物。免疫抑制治疗的影响尚不清楚。
