2018년 개정 지침에 따른 특발성 폐섬유증의 진단

권병수, 송진우
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引用次数: 1

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by worsening lung function and dyspnea. The prognosis of IPF patients is poor, as median survival is approximately 3 years. However, recently developed IPF-specific therapies have shown improved efficacies in terms of reducing lung function decline and mortality. Therefore, the early recognition and accurate diagnosis of IPF are crucial. In 2018, new guidelines for the diagnosis of IPF were published by the Fleischner Society and by the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society (ATS/ERS/JRS/ALAT). Both guidelines emphasize the need for a thorough history taking and physical examination to exclude an alternative diagnosis, such as exposure-related or connective tissue disease. The most informative initial examination is high-resolution computed tomography, the results of which can indicate the need for bronchoalveolar lavage or surgical lung biopsy, based on a multidisciplinary discussion of the findings and the patient’s clinical condition. A multidisciplinary discussion of the clinico-radiologic-pathologic findings is currently the gold standard in the diagnoisis of IPF and will allow the more effective and timely treatment of these patients. (Korean J Med 2019;94:173-181)
根据2018年修订方针特发性肺纤维症的诊断
特发性肺纤维化(IPF)是一种以肺功能恶化和呼吸困难为特征的进行性纤维化间质性肺疾病。IPF患者的预后较差,中位生存期约为3年。然而,最近开发的ipf特异性疗法在减少肺功能下降和死亡率方面显示出改善的疗效。因此,IPF的早期识别和准确诊断至关重要。2018年,Fleischner学会和美国胸科学会/欧洲呼吸学会/日本呼吸学会/拉丁美洲胸科学会(ATS/ERS/JRS/ALAT)发布了新的IPF诊断指南。两份指南都强调有必要进行全面的病史记录和体格检查,以排除其他诊断,如暴露相关疾病或结缔组织疾病。最重要的初步检查是高分辨率计算机断层扫描,其结果可以指示是否需要支气管肺泡灌洗或手术肺活检,这是基于对结果和患者临床状况的多学科讨论。临床-放射-病理结果的多学科讨论是目前诊断IPF的金标准,将允许对这些患者进行更有效和及时的治疗。(韩国医学杂志2019;94:173-181)
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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