Recognizing progressive pulmonary fibrosis: Diagnostic challenges and clinical tools

Song Jin Woo
Asan Medical Centre, Seoul, Korea

Progressive pulmonary fibrosis (PPF) is a clinical phenotype characterized by the progression of fibrotic lung deterioration in various underlying interstitial lung diseases (ILDs), even with standard treatment. Recognizing PPF is critical for timely intervention, yet it can be challenging due to its heterogeneous presentation and variable rates of fibrosis progression.

Key diagnostic elements include a thorough assessment of clinical decline evidenced by worsening dyspnea or cough. Significant physiological deterioration is also essential and is typically manifested by a decline in forced vital capacity (FVC) or diffusing capacity of the lung for carbon monoxide (DLCO). Radiological progression on high-resolution computed tomography (HRCT) is also essential and often shows increased reticulation, honeycombing, or traction bronchiectasis. These parameters form the basis for recognizing PPF, as recently emphasized in consensus statements and guidelines.

Clinical tools and strategies that facilitate the timely recognition of PPF include vigilant monitoring of lung function tests, serial HRCT imaging, and comprehensive symptom assessment. Multidisciplinary discussion (MDD) is emphasized as essential for integrating clinical, physiological, and radiological data in order to confirm the diagnosis of PPF and guide therapeutic decisions. Early recognition is crucial because specific antifibrotic therapies (e.g., nintedanib) have been shown to slow PPF progression. Starting these therapies early after diagnosis may improve lung function preservation and patient prognosis. This lecture aims to raise clinician awareness and provide a framework for accurately and promptly diagnosing PPF, ultimately improving patient management and outcomes.