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成功运用强化免疫抑制疗法治疗一名系统性红斑狼疮患者同时出现的脑部病变和肺动脉高压。

Successful use of intensive immunosuppressive therapy for treating simultaneously occurring cerebral lesions and pulmonary arterial hypertension in a patient with systemic lupus erythematosus.

作者信息

Watanabe Ryu, Fujii Hiroshi, Shirai Tsuyoshi, Saito Shinichiro, Hatakeyama Akira, Sugimura Koichiro, Fukumoto Yoshihiro, Ishii Tomonori, Harigae Hideo

机构信息

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2014;53(6):627-31. doi: 10.2169/internalmedicine.53.0514.

DOI:10.2169/internalmedicine.53.0514
PMID:24633036
Abstract

A 59-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE) was admitted to our hospital due to paralysis in all of her limbs. The patient presented with dysarthria, cerebellar ataxia and hypoxia. Magnetic resonance imaging (MRI) revealed vasogenic edema in the brain stem and the cerebellum. She was diagnosed with neuropsychiatric lupus syndrome (NPSLE) and pulmonary arterial hypertension (PAH), and was successfully treated using immunosuppressive therapy. To our knowledge, this is the first reported case of simultaneously developing NPSLE and PAH.

摘要

一名59岁的女性,此前已被诊断为系统性红斑狼疮(SLE),因四肢瘫痪入住我院。患者出现构音障碍、小脑共济失调和低氧血症。磁共振成像(MRI)显示脑干和小脑存在血管源性水肿。她被诊断为神经精神性狼疮综合征(NPSLE)和肺动脉高压(PAH),并通过免疫抑制治疗成功治愈。据我们所知,这是首例同时发生NPSLE和PAH的报告病例。

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引用本文的文献

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Autoimmune disease mouse model exhibits pulmonary arterial hypertension.自身免疫性疾病小鼠模型表现出肺动脉高压。
PLoS One. 2017 Sep 19;12(9):e0184990. doi: 10.1371/journal.pone.0184990. eCollection 2017.
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Cerebellar ataxia and obstructive hydrocephalus, rare neurologic presentations in patients with systemic lupus erythematosus.
小脑共济失调和阻塞性脑积水,系统性红斑狼疮患者罕见的神经系统表现。
Rheumatol Int. 2017 Nov;37(11):1917-1930. doi: 10.1007/s00296-017-3773-7. Epub 2017 Jul 13.
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Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide.糖皮质激素联合环磷酰胺治疗血管扩张剂抵抗的混合性结缔组织病相关肺动脉高压
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