RISS 검색 - 국내학술지논문 상세보기

다국어 초록 (Multilingual Abstract)

Idiopathic pulmonary fibrosis (IPF) is characterized by chronic progressive parenchymal lung

fibrosis. Although extensive researches for IPF pathogenesis have been reported for several

decades, the precise mechanisms are still unknown and the specific treatments for elimination of

fibrosis and prolongation of survival are also still unknown. The role of inflammation as initial

insult of lung fibrosis is still debating by controversial results of animal model experiments. The

recent proposed mechanism for IPF is a dysregulation of epithelial-mesenchymal interactions

which have critical role in tissue repair process and fibrosis. This hypothesis suggests impaired

communications between epithelium and mesenchymal cells in terms of abnormal proliferation of

mesenchymal cells instead of normal proliferation of epithelium. At recent, epithelial

mesenchymal transition is regarded as an important source of myofiborblast which are major

cells producing extracellular matrix. Classical treatment agents including steroid are already

known to be ineffective in treatment of IPF, and also, IFN- one of newly emerging drug, is

proved to be ineffective in treatment of IPF. Now new drugs involved in the molecular levels of

signal transduction of fibrotic pathway, inhibition of various growth factors (TGF, CTGF, VEGF),

and direct inhibition of fibrotic cytokines are under investigated in animal experiments and human

clinical studies. Further studies should be focused on the evaluation of precipitating factors,

genetic markers, drugs for inhibiting specific molecules responsible for lung fibrosis, and agents

for controlling ECM precipitation.

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