系统性红斑狼疮并发血栓性血小板减少性紫癜患者神经系统表现

Clinical Feature of Neurological Manifestations of Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)并发血栓性血小板减少性紫癜(thrombosic thrombocytopenic purpura,TTP)患者神经系统表现的临床特点。方法回顾性分析2011年1月至2015年12月北京协和医院17例SLE并发TTP患者的神经系统临床及影像学特点。结果 17例患者中,16例(94%)有神经系统表现,其中59%(10/17)的患者表现为意识障碍,47%(8/17)为痫性发作,29%(5/17)为局灶症状,24%(4/17)为头痛;53%(9/17)的患者有两种以上神经系统表现。12例进行了头颅影像学检查的患者中,75%(9/12)的患者头颅CT或MRI异常,其中8例(89%)多部位受累,2例(22%)患者病灶分布符合可逆性后部白质脑病的特征,4例(44%)患者的病灶符合梗死表现。糖皮质激素冲击治疗8例,糖皮质激素冲击联合血浆置换9例,治疗有效率分别为50%(4/8)、78%(7/9),总体有效率65%(11/17),1例患者死亡,4例病情恶化出院,1例需要维持血液透析治疗。11例治疗有效患者中,2例患者遗留神经系统后遗症。结论 SLE并发TTP神经系统受累主要表现为意识障碍、痫性发作,可有局灶症状。多数患者有头颅CT或MRI异常,病灶多部位分布。治疗有效患者中少数患者有神经系统后遗症。

Objective To analyze the clinical features of neurological manifestations of thrombotic thrombocytopenic purpura associated with systemic lupus Erythematosus. Methods The neurological characteristics and brain imaging of 17 patients with thrombotic thrombocytopenic purpura associated with systemic lupus Erythematosus accepted of Peking Union Medical College Hospital from 2011 to 2015 were retrospectively analyzed. Results Neurological symptoms occurred in 94% （ 16/17 ） of cases. The neurological symptoms included disturbance of consciousness （59%, 10/17）, seizure （47%, 8/17）, focal abnormalities （29%, 5/17） and headache （24%, 4/17）. 75% （9/12） of cases presentedpositive results in brain CT or MR. Multiple areas were affected in the majority （89%, 8/9 ）. 22% （2/9） of cases had evidence of posterior reversible encephalopathy syndrome, and 44% （4/9） had ischemic lesions. Eight patients were given steroids pulse therapy and four of them recovered. Nine patients were treated with methylprednisolone pulse therapy combined with plasmapheresis and seven of them recovered. During the follow-up, 1 died, 4 gave up with exacerbated symptoms, 1 remained dialysis and 11 recovered. Of 11 patients who recovered, 2 had neurological deficit left. Conclusions The main neurological manifestations of TIP associated with SLE are disturbance of consciousness and seizure, and focal abnormalities can occur. The majority of cases have positive findings in brain CT or MR with multiple areas affected. 65% of cases recovered after therapy and the minority of them have neurological deficit left.