Progressive pulmonary fibrosis: an expert group consensus statement

Area of Science:

• Pulmonology
• Respiratory Medicine
• Interstitial Lung Diseases

Background:

• Progressive fibrosing interstitial lung diseases (F-ILDs) present heterogeneous clinical courses.
• Accurate diagnosis and individualized management are critical for F-ILDs.
• The term progressive pulmonary fibrosis (PPF) accurately reflects a common clinical challenge.

Purpose of the Study:

• To provide expert consensus guidance on the diagnosis, treatment, and monitoring of F-ILDs.
• To emphasize the importance of standardizing F-ILD definitions and accurate initial diagnosis.
• To highlight the role of antifibrotic therapy and tailored monitoring strategies for PPF.

Main Methods:

• Expert group consensus statement development.
• Review of current diagnostic and management strategies for F-ILDs.
• Discussion of risk stratification, monitoring, and therapeutic interventions.

Main Results:

• Standardized definitions and accurate diagnosis are paramount for F-ILD management.
• Case-by-case decisions for monitoring and management are essential due to disease variability.
• Early antifibrotic therapy is increasingly supported for progressive pulmonary fibrosis (PPF).

Conclusions:

• Expert guidance is provided for the diagnosis, treatment, and monitoring of F-ILDs.
• Recognition and management of PPF, especially after initial treatment failure, are emphasized.
• Considerations for pulmonary rehabilitation, oxygen, ventilation, and lung transplantation are addressed.