특발성 폐섬유증의 진단과 치료

초록

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.

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