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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 investigate the clinical features of the nervous system in patients with systemic lupus erythematosus (SLE) complicated with thrombocytopenic purpura thrombosis (TTP). Methods The clinical and imaging features of 17 patients with SLE complicated with TTP from January 2011 to December 2015 in Peking Union Medical College Hospital were analyzed retrospectively. Results Of the 17 patients, 16 (94%) had neurological manifestations. 59% (10/17) of them showed disturbance of consciousness, 47% (8/17) were seizures, 29% (5/17 ) Had focal symptoms, 24% (4/17) had headaches and 53% (9/17) had more than two neurological manifestations. Of the 12 patients who underwent cranial imaging examination, 75% (9/12) of the patients had abnormal CT or MRI craniocerebral lesions, of which 8 (89%) had multi-site involvement and 2 (22%) had focal lesions that were reversible Posterior white matter encephalopathy was characterized in 4 (44%) patients with lesions that met infarction performance. Eight patients were treated with glucocorticoid shock and 9 patients were treated with glucocorticoid shock and plasma exchange. The effective rates were 50% (4/8), 78% (7/9), and 65% (11/17), respectively. One patient died, four were exacerbated and one was required to maintain hemodialysis. Of the 11 patients who were effectively treated, 2 had neurological sequelae. Conclusion SLE concurrent TTP nervous system involvement mainly manifested as disturbance of consciousness, epileptic seizures, may have focal symptoms. Most patients have skull CT or MRI abnormalities, multi-site distribution of lesions. A small number of patients in the treatment-effective group have neurological sequelae.