Progressive pulmonary fibrosis (PPF) is a clinical phenotype observed across various interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis, characterized by worsening fibrosis on high-resolution computed tomography (HRCT), progressive decline in lung function, and deteriorating respiratory symptoms. Despite shared pathophysiological mechanisms, the rate of disease progression and clinical outcomes vary substantially according to each subtype of PPF. Key predictors of poor prognosis include advanced age, male sex, rapid lung function decline, hypoxemia, pulmonary hypertension, and radiologic features such as a usual interstitial pneumonia pattern and traction bronchiectasis. Genetic factors, particularly telomere shortening and mutations in telomere-related genes, have emerged as important determinants of prognosis and therapeutic response. Acute exacerbation of PPF is associated with poor short-term prognosis representing a major cause of morbidity and mortality in PPF. The introduction of antifibrotic therapies has significantly altered the management of PPF by slowing lung function decline. However, their impact on long-term survival remains under investigation.
Joo Hun Park (Fri,) studied this question.