OBJECTIVES: Progressive fibrosing lung diseases (PF-ILDs), including idiopathic pulmonary fibrosis (IPF), share common pathophysiologic characteristics and are associated with substantial morbidity and mortality. Antifibrotic (AF) therapy aims to manage symptoms and slow disease progression, thereby improving morbidity and life expectancy. However, uptake and adherence is variable. An understanding of the burden of PF-ILD in the era of AF therapy is required to inform payers of clinical unmet needs. This targeted literature review aims to identify the humanistic (health-related quality of life [HRQoL]) and economic burden of PF-ILD and IPF.

METHODS: Electronic databases (Embase, MEDLINE, the Cochrane Library) were searched. Studies published in English between 2017–2022 evaluating HRQoL, healthcare resource use, or cost impact in adult patients with a diagnosis of IPF or PF-ILD and their caregivers were included.

RESULTS: Twenty-five studies were included: fourteen reporting humanistic burden and eleven reporting economic burden, with the majority reporting data on IPF. IPF led to impairment in patient HRQoL as assessed by multiple disease-specific and generic patient-reported outcome instruments, particularly for mobility, activity, pain, anxiety, and depression domains. Increasing disease severity and comorbidities were associated with significant worsening of HRQoL, increased risk of hospitalization, and higher healthcare costs. Hospitalization rates ranged from 21.7–78.5%, with high levels of primary care, specialist care and medication resource use. For one-third of patients, ability to work was impaired. Delayed diagnosis and delayed initiation of AF treatment was associated with increased healthcare costs.

CONCLUSIONS: IPF is associated with a substantial humanistic and economic burden driven by disease severity and comorbidities. Slowing disease progression with early AF treatment initiation whilst managing symptoms may reduce the overall burden of disease. Additional real-world evidence (RWE) assessing burden in treatment-naive versus AF-treated patients and further RWE in patients with non-IPF PF-ILD are required to inform treatment decisions.