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神经结节病

Neurosarcoidosis.

作者信息

Ungprasert Patompong, Matteson Eric L

机构信息

Division of Rheumatology, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, 200 First Avenue Southwest, Rochester, MN 55905, USA; Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Prannok Road, Bangkok 10700, Thailand.

Division of Rheumatology, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, 200 First Avenue Southwest, Rochester, MN 55905, USA; Division of Epidemiology, Department of Health Science Research, Mayo Clinic College of Medicine and Science, 200 First Avenue Southwest, Rochester, MN 55905, USA.

出版信息

Rheum Dis Clin North Am. 2017 Nov;43(4):593-606. doi: 10.1016/j.rdc.2017.06.008. Epub 2017 Aug 23.

DOI:10.1016/j.rdc.2017.06.008
PMID:29061245
Abstract

Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis. Cranial neuropathy and meningeal involvement are the most common manifestations, but any part of the nervous system can be affected. Definite diagnosis requires the presence of noncaseating granuloma in the nervous system, although histopathologic confirmation is often not obtainable. Moderate to high dose of glucocorticoids is the main therapy for neurosarcoidosis. Relapse often occurs after the dose of glucocorticoids is tapered down, often necessitating the use of steroid-sparing immunosuppressive agents.

摘要

神经结节病发生于3%至10%的结节病患者中。颅神经病变和脑膜受累是最常见的表现,但神经系统的任何部位都可能受到影响。明确诊断需要在神经系统中存在非干酪样肉芽肿,尽管组织病理学确诊往往无法实现。中高剂量的糖皮质激素是神经结节病的主要治疗方法。糖皮质激素剂量减小时常常会复发,这通常需要使用能减少激素用量的免疫抑制剂。

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