OBJECTIVES: Patients with PPF experience debilitating and worsening symptom burden leading to significant hospitalizations, high costs and deterioration in health-related quality of life (HRQoL) despite treatment. The SLRs aim to describe the humanistic and economic burden associated with PPF.

METHODS: Two SLRs adhering to Cochrane/PRISMA guidelines were conducted between January 2014-September 2023 using Embase, MEDLINE, EconLit, Cochrane and grey literature. All prices were inflated to 2024, Euros.

RESULTS: Five studies assessed the humanistic burden, utilizing HRQoL measures including SGRQ (n=2), K-BILD (n=2), C-VAS, CAT, PSQI, and Delphi questionnaire (n=1). There was a decline in patients’ HRQoL from baseline to follow up as measured by K-BILD [-1.9 mean change at 12 months], SGRQ [5.8 mean change at 24 months], PSQI [0.9 mean change at 12 months], and CAT [2.0 mean change at 24 months]. Despite a decline in other HRQoL measures over time, a decrease in cough was observed as measured by C-VAS [-0.7 mean change over 6 months]. Results from a Delphi questionnaire indicated that 85-90% of panelists agreed that PPF impacted unpaid caregivers’ QoL in terms of sleep and health, daily activities, emotional well-being, social life, and finances. Nine studies assessing the economic burden due to PPF indicated a substantial burden of the disease on the healthcare systems. The mean total direct per-patient per-year (PPPY) costs (varying types of direct costs) for managing PPF ranged from €35,607 to €86,293 in the US (n=4) and from €16,810 (Greece) to €86,458 (France) across Europe (n=3). There was an increase in the annual management costs on follow-up in the US while a decrease in the follow-up annual costs was observed in Europe. Hospitalization emerged as a key cost driver for economic burden across the studies.

CONCLUSIONS: These findings suggest that patients with PPF face significant humanistic and economic burden, with costs primarily driven by hospitalizations.