Cough burden in progressive pulmonary fibrosis: a systematic review with narrative synthesis

Cough is a common and burdensome symptom in patients with progressive pulmonary fibrosis (PPF), yet existing evidence remains fragmented. This systematic review provides a synthesis of available evidence on cough in PPF, specifically examining its association with health-related quality of life (HRQoL), the instruments used to assess cough, current treatment evidence, and identifies priorities for future research.

We searched the Embase, PubMed, Cochrane, and Web of Science (WOS) databases from their inception to January 12, 2026, for studies focusing on the burden of cough in patients with PPF. We extracted information on study details, participant features, and outcome measures, and assessed the risk of bias in the included studies. A narrative synthesis was then performed to summarize the evidence.

Of 11,683 records, 9 studies met inclusion criteria. Cough was most frequently assessed using VAS and LCQ, while HRQoL was primarily measured with SGRQ and L-PF. Two studies highlighted wearable devices for objective cough monitoring. Antifibrotic therapy showed no consistent or statistically significant benefit on cough in descriptive synthesis. Qualitative studies revealed substantial cough‑related burden, including daily activity limitations, fatigue, and social embarrassment. Regarding economic impact, although no dedicated studies exist, one study indicated that cough severity was associated with workplace productivity loss.

Cough negatively impacts HRQoL in PPF, but evidence is limited by heterogeneous tools and lack of economic studies. Standardized assessment and targeted research on cough management and its economic impact are urgently needed.