Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center.
Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center.
复制标题
DOI：
10.3346/jkms.2016.31.11.1846
复制
发表时间：
2016-11
影响因子：
4.5
通讯作者：
Jeon CH
中科院分区：
文献类型：
--
作者：
Jeon CH
复发性多软骨炎(RP)是一种以耳、鼻、外周关节和气管支气管树等软骨结构炎症为特征的自身免疫性疾病。它很少累及中枢神经系统(CNS)，但RP的CNS并发症的诊断具有挑战性，因为它可以表现出不同的临床特征。本文报告3例累及中枢神经系统的复发性多软骨炎，具有不同的临床表现和病程。第一例患者表现为菱形脑炎，导致脑水肿和死亡。第二例患者因边缘脑炎而出现急性认知功能障碍。他接受了类固醇脉冲治疗，并痊愈了，没有后遗症。第三名患者患有无菌性脑膜炎，表现为痴呆症，对类固醇和免疫抑制剂无效。我们还复习了有关RP中枢神经系统并发症的文献。
Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.
登录
查看更多内容
影响因子：
3.4
作者：
Choi, Hyuk Jai;Lee, Hee Jung
通讯作者：
Lee, Hee Jung
影响因子：
3.4
作者：
Hsu, KC;Wu, YR;Tang, LM
通讯作者：
Tang, LM
影响因子：
3.5
作者：
Mihara, T.;Ueda, A.;Mutoh, T.
通讯作者：
Mutoh, T.
影响因子：
3.4
作者：
Yang, SM;Chou, CT
通讯作者：
Chou, CT
影响因子：
1.2
作者：
Yaguchi, Hiroaki;Tsuzaka, Kazufumi;Sasaki, Hidenao
通讯作者：
Sasaki, Hidenao