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특발성 폐 섬유화증의 병인 및 최신 진단 기준

Pathogenesis and New Diagnosis Guideline of Idiopathic Pulmonary Fibrosis

어수택
대한내과학회 (The Korean Association Of Internal Medicine)|2013.04|대한내과학회지 (The Korean Journal of Medicine)|제84권 제4호|pp. 481-488|DOI http://dx.doi.org/10.3904/kjm.2013.84.4.481

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참고문헌

키워드

진단Diagnosis병인PathogenesisMyofibroblastsIdiopathic pulmonary fibrosis특발성 폐 섬유화증근육섬유모세포
주제분류내과학

초록

현재까지도 IPF의 발생 원인은 알려져 있지 않지만, 여러연구의 발달로 병인을 밝히는 데 많은 발전이 있었다. 특히후생학 연구의 발달로 IPF의 병인을 DNA부터 전사 후(post-transcription)의 변화까지 알 수 있게 되었다. 하지만 섬유화에 가장 중요한 TGF-β 활성화만으로는 IPF의 병인을 모두 설명할 수 없으며, 섬유화에 이르게 하는 여러 세포와 단백질이 상호 활성화시키면서 섬유화가 계속 진행하는 것으로 보이며, 이에 대한 연구가 진행되어야 할 것이다. 병인과 달리 진단은 많은 발전이 있었다. 특히 최근에 발표된 지침을 통하여 폐 조직 검사 없이도 진단할 수 있는 근거를 마련하였으며, IPF의 진단을 위하여 관계하는 과간의상의를 통하여 진단할 수 있게 되었다.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrotic lung disease in many patients. In spite of extensive research for many decades, the exact pathogenesis of IPF is unknown. At recent, the role of alveolar epithelial cells has been focused in the initiation of IPF in terms of epithelial-mesenchymal transition, dysregulated Wnt signaling, and activation of transforming growth factor-β (TGF-β). The resulting excess collagen deposition and destruction of lung architecture by myofibroblasts and fibroblastic foci leads to the development of IPF. IPF can be diagnosed by typical high resolution chest tomogram (HRCT) or by multidisciplinary discussion based on the new guideline published on 2010.

참고문헌 (42)

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