Navigating Progressive Pulmonary Fibrosis: Diagnostic Precision and Management Strategies

Progressive Pulmonary Fibrosis (PPF), with its intricate clinical challenges, continues to redefine the landscape of pulmonary medicine. The dynamic criteria used to diagnose PPF and the concurrent evolution of management strategies are transforming patient care, with ongoing studies bringing new insights into this ever-changing field.

The latest criteria for diagnosing PPF emphasize a combination of worsening respiratory symptoms, decline in pulmonary function tests, and radiological progression, as outlined by the 2022 ATS/ERS/JRS/ALAT clinical practice guideline. The same interplay of symptoms and test results that helps diagnose PPF also guides tailored management plans, with a recent clinical update offering useful context without replacing guideline criteria. This approach centers diagnosis on longitudinal symptoms, physiology, and imaging changes.

Diagnostic pathways for PPF not only clarify patient symptoms but also streamline intervention frameworks. Changes in pulmonary function, specifically in FVC and DLCO (with progression often defined by a relative FVC decline of ≥10%, or 5–9% accompanied by symptomatic or radiologic worsening), along with HRCT progression, are central to guiding treatment decisions, as summarized in a recent review of antifibrotic therapy. These metrics help track disease course and inform adjustments in therapeutic strategies.

Antifibrotic therapy features prominently in current management discussions. Nintedanib has randomized-trial evidence supporting a reduction in the rate of FVC decline in non-IPF fibrosing ILDs with a progressive phenotype, whereas pirfenidone data are more limited and disease-specific. These agents may be incorporated into comprehensive care alongside individualized symptom monitoring and supportive measures, with decisions guided by tolerability and patient-specific risk–benefit considerations.

Such findings may reshape clinician approaches to long-term disease management. For patients experiencing persistent dyspnea, these diagnostic tools validate their experiences. Antifibrotic therapies allow for targeted approaches to care, emphasizing the necessity of continuous research and adaptation of new treatments.

Managing PPF remains daunting, especially when treatment response varies significantly. Because pulmonary function tests and imaging changes closely track disease progression, timely interventions are often feasible. If imaging reveals further fibrosis, additional evaluation for antifibrotic therapy may be warranted. As these tools develop, the paradigm of treating PPF may continue to shift toward more precisely targeted and efficacious outcomes for patients.

Despite technological advancements, some aspects of disease management remain unclear. Not every patient presents with typical symptoms, illustrating the need for flexible diagnostic strategies. Building on PFT-anchored monitoring, tools such as home spirometry and app-based symptom tracking can complement clinic-based thresholds and imaging review, informing when to adjust therapy. The next step is to incorporate these criteria and tools into routine care to enhance efficacy.

Key Takeaways:

• The latest diagnostic criteria integrate symptoms, function, and imaging to ensure thorough patient evaluations.
• Antifibrotic therapy—particularly nintedanib based on randomized evidence—can slow FVC decline in progressive fibrosing ILDs; use is individualized.
• Patient-centered strategies emphasize symptom monitoring and tailored therapeutic approaches.