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结节病。

Sarcoidosis.

机构信息

Research and Innovation Group in Autoimmune Diseases, Sanitas Digital Hospital, Hospital-CIMA-Centre Mèdic Millenium Balmes Sanitas, Barcelona, España; Línea de Investigación en Sarcoidosis del GEAS, Sociedad Española de Medicina Interna (SEMI), Madrid, España.

Línea de Investigación en Sarcoidosis del GEAS, Sociedad Española de Medicina Interna (SEMI), Madrid, España; Servicio de Medicina Interna, Hospital San Rafael, La Coruña, España.

出版信息

Med Clin (Barc). 2022 Aug 26;159(4):195-204. doi: 10.1016/j.medcli.2022.03.009. Epub 2022 Jun 6.

DOI:10.1016/j.medcli.2022.03.009
PMID:35680449
Abstract

Sarcoidosis is a systemic autoimmune disease that is associated with the development of non-caseating granulomas. The disease especially affects the lymph nodes, lungs, skin and eyes. It is an infrequent but not rare disease, especially in northern Europe, the United States and India. Sarcoidosis affects more women and is diagnosed between the ages of 30 and 50. Etiopathogenically, it is closely linked to environmental factors, especially occupational exposures. Clinically, it stands out for a wide variety of presentation phenotypes (casual diagnosis, involvement of a single organ or systemic presentation). The diagnosis of sarcoidosis is complex and requires the integration of clinical, analytical, radiological, and histolopathogical data carried out by multidisciplinary clinical units. The evolution of the disease is variable, as is the indication for systemic treatment, based on the use of corticosteroids as first-line option, the use of immunosuppressants as second-line therapy, and anti-TNF agents in severe and/or refractory cases.

摘要

结节病是一种系统性自身免疫性疾病,与非干酪样肉芽肿的发展有关。这种疾病特别影响淋巴结、肺部、皮肤和眼睛。它是一种不常见但并不罕见的疾病,特别是在北欧、美国和印度。结节病更多地影响女性,诊断年龄在 30 至 50 岁之间。病因上,它与环境因素密切相关,特别是职业暴露。临床上,它以多种多样的表现表型为特征(偶然诊断、单一器官受累或全身表现)。结节病的诊断较为复杂,需要多学科临床单位整合临床、分析、放射和组织病理学数据。疾病的演变是多变的,需要全身治疗的指征也是如此,基于一线选择使用皮质类固醇,二线治疗使用免疫抑制剂,以及在严重和/或难治性病例中使用抗 TNF 药物。

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