Progressive pulmonary fibrosis: the importance of identification and intervention

TL;DR

Identifying progressive pulmonary fibrosis early is crucial for timely treatment and better patient outcomes.

Contribution

Highlights the importance of flexible criteria for identifying progressive pulmonary fibrosis in clinical practice.

Findings

PPF is associated with high morbidity and mortality regardless of its definition.

Prompt identification of PPF enables timely treatment escalation and supportive care.

Future research may allow earlier treatment for patients at risk of progression.

Abstract

The concept of progressive pulmonary fibrosis (PPF) was developed to facilitate the identification of patients with an interstitial lung disease (ILD) that is worsening and requires treatment. Various criteria have been proposed to identify PPF, generally based on a deterioration in forced vital capacity alone or with worsening of respiratory symptoms and/or radiological abnormalities. All these criteria are imperfect and based on a limited evidence base. PPF, however it is defined, is associated with high morbidity and mortality. In clinical practice, flexibility is needed in defining ILD progression given differences in the frequencies and methodologies used to monitor patients’ disease. Prompt identification of PPF is important to enable timely initiation or escalation of treatment to slow progression of lung fibrosis, consider eligibility for lung transplantation and provide supportive care as needed. In future, earlier treatment of patients at risk of progression may be possible to improve outcomes for patients.

Prompt identification of disease progression in patients with interstitial lung disease is important so that initiation or escalation of treatment can be considered and supportive care provided as needed.
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