특발성 폐섬유증의 진단과 치료.

Purpose: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown etiology that primarily affects older adults. It is characterized by worsening dyspnea, declining lung function, and poor prognosis. This review summarizes current approaches to IPF...

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Publicado en:Journal of the Korean Medical Association / Taehan Uisa Hyophoe Chi Vol. 69; no. 6; pp. 460 - 468
Autor principal: Yoo, Jung-Wan
Formato: algorithm review tables/charts Journal Article
Publicado: Korean Medical Association Jun2026
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jun2026
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      pub: Korean Medical Association
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        atl: 특발성 폐섬유증의 진단과 치료.
      aug:
        au: Yoo, Jung-Wan
        affil: Department of Internal Medicine, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
      sug:
        subj:
          Idiopathic Pulmonary Fibrosis Diagnosis
          Idiopathic Pulmonary Fibrosis Therapy
          Multidisciplinary Care Team
          Tomography, X-Ray Computed
          Biopsy Methods
          Rehabilitation, Pulmonary
          Oxygen Therapy
          Lung Transplantation
          Antifibrotic Agents Therapeutic Use
          Vital Capacity
      ab: Purpose: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown etiology that primarily affects older adults. It is characterized by worsening dyspnea, declining lung function, and poor prognosis. This review summarizes current approaches to IPF diagnosis and treatment. Current concepts: Diagnosis requires a multidisciplinary approach integrating clinical, radiological, and, when necessary, histopathological findings. A key feature is the usual interstitial pneumonia pattern on high-resolution computed tomography and/or lung biopsy. Pirfenidone and nintedanib appear to slow the decline in lung function, including forced vital capacity (FVC), and are recommended as first-line therapies. Clinical trials of novel agents are ongoing, with several recent trials yielding promising findings. Non-pharmacological management, including pulmonary rehabilitation, oxygen therapy, and symptom control, is essential for comprehensive care. Lung transplantation remains the only curative option and should be considered in eligible patients. Discussion and conclusion: Early, accurate IPF diagnosis is essential and requires a multidisciplinary approach that integrates clinical, radiological, and histopathological findings, as outlined in the 2018 and 2022 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guidelines. Pirfenidone and nintedanib remain the cornerstone antifibrotic agents for pharmacological treatment, slowing FVC decline and reducing the risk of acute exacerbation. Emerging agents, including nerandomilast and inhaled treprostinil, have shown promise in recent phase 3 clinical trials. Non-pharmacological strategies, including pulmonary rehabilitation, oxygen therapy, and palliative care, are integral to comprehensive management. Despite these advances, IPF remains a progressive and fatal disease, underscoring the need for continued research into novel therapeutic strategies.
      pubtype: Academic Journal
      doctype:
        algorithm
        review
        tables/charts
        Journal Article
      ougenre: Article
    language: Korean
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