Suppr超能文献

结节病

Sarcoidosis.

作者信息

Salah S, Abad S, Monnet D, Brézin A P

机构信息

Department of Ophthalmology, hôpital Cochin, Assistance publique-Hôpitaux de Paris, université Paris Descartes, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.

Department of Internal Medicine, hôpital Avicenne, Assistance publique-Hôpitaux de Paris, 125, rue de Stalingrad, 93000 Bobigny, Paris, France.

出版信息

J Fr Ophtalmol. 2018 Dec;41(10):e451-e467. doi: 10.1016/j.jfo.2018.10.002. Epub 2018 Nov 16.

Abstract

Sarcoidosis is a systemic granulomatosis characterized by the formation of epithelioid and giant cell granulomas without caseous necrosis. To make the diagnosis, it is necessary to prove systemic granulomatosis involving at least two organs; but in practice, a combination of clinical, paraclinical and histologic findings is used. It affects predominantly women with a bimodal age distribution: 25-29 years and 65-69 years. The most commonly affected organs are the mediastinal lymphatic system, lungs, skin and eyes. Ophthalmological involvement is present in 20 to 50% of cases. The typical ocular presentation is that of granulomatous uveitis associated with venous retinal vasculitis and lesions of peripheral multifocal choroiditis. This ophthalmological presentation, although very evocative, is not always associated with systemic disease. The diagnosis of ocular sarcoidosis is then presumed in the absence of histological evidence. Algorithms combining ophthalmological and systemic signs have been proposed in cases of isolated uveitis. They make it possible to establish the diagnosis of ocular sarcoidosis with various levels of probability. The absence of significant granulomas on a systemic level during primary ocular involvement remains the main hypothesis to explain these diagnostic difficulties. Treatment is well described, as the uveitis of sarcoidosis is most often steroid responsive. In the case of corticosteroid-dependent uveitis, the first-line immunosuppressant remains methotrexate. The use of anti-tumor necrosis factor alpha is an interesting alternative in patients whose ocular sarcoidosis is refractory to conventional immunosuppressants.

摘要

结节病是一种全身性肉芽肿病,其特征是形成上皮样和巨细胞肉芽肿,无干酪样坏死。要做出诊断,必须证明全身性肉芽肿病累及至少两个器官;但在实践中,会综合临床、辅助检查和组织学检查结果进行诊断。该病主要影响女性,年龄分布呈双峰型:25 - 29岁和65 - 69岁。最常受累的器官是纵隔淋巴系统、肺、皮肤和眼睛。20%至50%的病例存在眼部受累。典型的眼部表现是肉芽肿性葡萄膜炎,伴有视网膜静脉血管炎和周边多灶性脉络膜炎病变。这种眼部表现虽然很有提示性,但并不总是与全身性疾病相关。在没有组织学证据的情况下,则推测为眼部结节病。对于孤立性葡萄膜炎病例,已经提出了结合眼部和全身体征的诊断方法。它们能够以不同的概率水平确诊眼部结节病。原发性眼部受累时全身无明显肉芽肿仍然是解释这些诊断困难的主要假说。治疗方法已有详细描述,因为结节病性葡萄膜炎通常对类固醇治疗有反应。对于依赖皮质类固醇的葡萄膜炎病例,一线免疫抑制剂仍然是甲氨蝶呤。对于眼部结节病对传统免疫抑制剂难治的患者,使用抗肿瘤坏死因子α是一种有趣的替代方法。

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