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2018년 개정 지침에 따른 특발성 폐섬유증의 진단

New Diagnostic Guidelines for Idiopathic Pulmonary Fibrosis

대한내과학회지 2019년 94권 2호 p.173 ~ 181
권병수 ( Kwon Byoung-Soo ) - 서울대학교 의과대학 분당서울대학교병원 호흡기내과

송진우 ( Song Jin-Woo ) - 울산대학교 의과대학 서울아산병원 호흡기내과

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

키워드

Idiopathic pulmonary fibrosis; Diagnosis; Guideline
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