Suppr超能文献

免疫介导性内耳疾病:10年经验

Immune-mediated inner ear disease: 10-year experience.

作者信息

Broughton Shelley S, Meyerhoff William E, Cohen Stanley B

机构信息

Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA.

出版信息

Semin Arthritis Rheum. 2004 Oct;34(2):544-8. doi: 10.1016/j.semarthrit.2004.07.001.

Abstract

BACKGROUND

Autoimmune inner ear disease (AIED) was first described in 1979 and the disease has become more widely recognized over the last decade. Limited information is available regarding clinical features of the disease, disease course, and response to treatment.

OBJECTIVE

To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome.

METHODS

A retrospective chart review was conducted on all patients considered to have AIED by the Otolaryngology division physicians and on all patients positive for antibody testing to inner ear antigens from 1990 to 1999. Patients who were antibody positive with a clinical diagnosis of AIED were included in this review.

RESULTS

Patients with AIED presented with rapidly progressive, frequently bilateral (79%), often fluctuating sensironeural hearing loss. Mean age at presentation was 50 years (22-80) with no gender predilection. Tinnitus (83%), vestibular complaints (79%), and Menieres (50%) were common concomitant symptoms along with hearing loss. Seven of 42 (17%) of the patients had evidence for other systemic autoimmune disorders. In 4 of the patients the onset of vestibuloauditory complaints preceded the diagnosis of autoimmune disorder. Thirty-three of 42 demonstrated antibodies to inner ear antigens but other autoantibodies were infrequent except in patients with systemic autoimmune disorders. Twenty-three of 33 (70%) of patients treated with corticosteroids improved clinically, often short-term. Sixteen patients received treatment with other immunosuppressive drugs including methotrexate, cyclophosphamide, azathioprine, mycophenolic mofetil, and intravenous immunoglobulin (IVIg) generally with a limited modest response. Using clinical trial criteria for response, only 5/35 (14%) demonstrated improvement over a mean 34.4 months follow-up.

CONCLUSIONS

Immune-mediated inner ear disease is not a uniform disease with simple diagnosis or treatment. The course of the disease often results in significant long-term disability due to hearing loss and response to aggressive immunosuppression including corticosteroids is poor.

摘要

背景

自身免疫性内耳疾病(AIED)于1979年首次被描述,在过去十年中该疾病得到了更广泛的认识。关于该疾病的临床特征、病程及治疗反应的信息有限。

目的

分析42例有免疫介导性内耳疾病记录患者的数据,以进一步明确该综合征。

方法

对耳鼻喉科医生认为患有AIED的所有患者以及1990年至1999年内耳抗原抗体检测呈阳性的所有患者进行回顾性病历审查。本综述纳入临床诊断为AIED且抗体呈阳性的患者。

结果

AIED患者表现为快速进行性、常为双侧性(79%)、常波动的感音神经性听力损失。发病时的平均年龄为50岁(22 - 80岁),无性别倾向。耳鸣(83%)、前庭症状(79%)和梅尼埃病(50%)是与听力损失常见的伴随症状。42例患者中有7例(17%)有其他系统性自身免疫性疾病的证据。4例患者的前庭听觉症状发作先于自身免疫性疾病的诊断。42例中有33例显示对内耳抗原有抗体,但除了患有系统性自身免疫性疾病的患者外,其他自身抗体很少见。33例接受皮质类固醇治疗的患者中有23例(70%)临床症状改善,通常为短期改善。16例患者接受了包括甲氨蝶呤、环磷酰胺、硫唑嘌呤、霉酚酸酯和静脉注射免疫球蛋白(IVIg)在内的其他免疫抑制药物治疗,总体反应有限且适度。根据临床试验的反应标准,在平均34.4个月的随访中,只有5/35(14%)显示有改善。

结论

免疫介导性内耳疾病并非一种诊断或治疗简单的统一疾病。由于听力损失,该疾病的病程常常导致严重的长期残疾,且包括皮质类固醇在内的积极免疫抑制治疗反应不佳。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验