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《韩国间质性肺疾病诊断与管理指南:结节病》

Korean Guidelines for the Diagnosis and Management of Interstitial Lung Diseases: Sarcoidosis.

作者信息

Lee Eun Joo, Jegal Yangjin, Park Dong Won, Park Jimyung, Myong Jun-Pyo, Lee Ji-Hyun, Kang Bo Hyoung

机构信息

Division of Respiratory, Critical Care Medicine and Allergy, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea.

Division of Pulmonary and Critical Care Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Republic of Korea.

出版信息

Tuberc Respir Dis (Seoul). 2025 Jul;88(3):488-503. doi: 10.4046/trd.2024.0202. Epub 2025 Apr 1.

Abstract

Sarcoidosis is a granulomatous inflammatory disease of unknown etiology that can affect the entire body, but its respiratory tract involvement is most common. In radiological findings, bilateral hilar and mediastinal lymph node enlargement is the most common finding, and when lung parenchyma is involved, findings such as micronodules, ground-glass shadows, reticular shadows, and fibrosis are seen. Biopsies for histological diagnosis are mainly performed on lymph nodes or lungs, and mediastinal lymph node biopsies are done via endobronchochial ultrasound-guided fine needle aspiration. Pathological findings are characterized by non-caseating, non-necrotizing granulomas, and in Korea, differentiation from tuberculosis is especially important. The natural history of sarcoidosis is very variable, with approximately two-thirds of subjects showing spontaneous remission and only 10% to 30% of patients showing a chronic or progressive form. The most important factor in determining treatment for sarcoidosis is whether there is a risk of death, organ failure, or loss of quality of life. Glucocorticosteroids are the mainstay of treatment, and if the disease progresses despite treatment, or if glucocorticosteroids cannot be reduced or cannot be used, immunosuppressants such as methotrexate and azathioprine can be considered. Response to glucocorticosteroids treatment is good, and most of the patients stabilize or improve, with a low mortality rate of around 3% to 5%.

摘要

结节病是一种病因不明的肉芽肿性炎症性疾病,可累及全身,但最常累及呼吸道。在影像学表现中,双侧肺门和纵隔淋巴结肿大是最常见的表现,当肺实质受累时,可见微结节、磨玻璃影、网状影和纤维化等表现。组织学诊断活检主要在淋巴结或肺部进行,纵隔淋巴结活检通过支气管内超声引导下细针穿刺进行。病理表现以非干酪样、非坏死性肉芽肿为特征,在韩国,与结核病的鉴别尤为重要。结节病的自然病程差异很大,约三分之二的患者可自发缓解,只有10%至30%的患者表现为慢性或进行性形式。决定结节病治疗的最重要因素是是否存在死亡、器官衰竭或生活质量下降的风险。糖皮质激素是治疗的主要药物,如果尽管进行了治疗疾病仍进展,或者如果糖皮质激素不能减量或不能使用,可以考虑使用甲氨蝶呤和硫唑嘌呤等免疫抑制剂。对糖皮质激素治疗的反应良好,大多数患者病情稳定或改善,死亡率较低,约为3%至5%。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/06ba/12235296/fc68e18beb94/trd-2024-0202f1.jpg

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