摘要目的 总结分析复发性多软骨炎合并中枢神经系统损害的临床特征.方法 分析5例复发性多软骨炎合并中枢神经系统病变的临床表现、脑脊液检查结果和头颅MRI资料,并与文献资料比较.采用t检验和Fisher确切概率法进行统计学分析.结果 本院205例复发性多软骨炎患者中5例(2.4％)出现中枢神经系统损害,其中女性3例,平均年龄(44±14)岁.5例中枢神经系统损害病例均起病急,于复发性多软骨炎活动期出现,均表现为脑膜脑炎,2例合并颅神经病变.脑脊液检查提示非特异炎性病变;头颅MRI提示脑组织病灶处长T2信号影.4例对糖皮质激素和(或)免疫抑制剂治疗反应良好.本研究患者较文献报道患者平均年龄低[分别为(44±14)岁,(58±11)岁,t=2.547,P＜0.05],在复发性多软骨炎和中枢神经系统损害表现方面差异无统计学意义.结论 中枢神经系统病变是复发性多软骨炎的罕见表现,多于复发性多软骨炎活动期急性发作,临床表现以脑膜脑炎/脑膜炎为主,MRI、脑脊液检查等有助于诊断,糖皮质激素联合免疫抑制剂治疗有效.

abstractsObjective To study the clinical profile of patients with central nervous system (CNS) involvement in relapsing polychondritis (RP).Methods Clinical data of five patients of RP with CNS lesions were collected,and compared with those from the literatures.T test and Fisher exact test were used for statistical analysis.Results Among 205 patients with RP in Peking Union Medical College Hospital,five cases (2.4％) had CNS damage.All 5 cases presented an active onset and occurred in the active phase of RP.All of them manifested as meningoencephalitis,complicating cranial neuropathies in 2 cases (2/5).Cerebrospinalfluid examination revealed non-specific meningeal inflammation,and magnetic resonance image (MRI) showed long T2 signals in brain lesions.Four patients (4/5) showed good response to high-dose glucocorticosteroid plus immunosuppressive agent combined therapy.The average age of our patients was younger than those in the literatures [(44± 14),(58± 11) years,respectively; t=2.547,P＜0.05],while other clinical features was not significantly different between the two groups.Conclusion CNS involvement is a rare condition in RP patients,and usually occurrs in the early course of active RP.Meningoencephalitis/ meningitis is the major clinical manifestations.MRI and cerebrospinal fluid examination may help to confirm the diagnosis.Treatment with corticosteroid and immunosuppressant can result in favorable response.