Global perspective on pulmonary fibrosis: epidemiology, regional variations, gaps in care and future directions

BACKGROUND: Pulmonary fibrosis (PF) caused by interstitial lung diseases (ILDs) affects up to 0.1% of the global population. There is growing global interest in PF, evident from a nearly threefold increase in PF-related research publications over the last 15 years. Idiopathic PF, connective tissue disease-related ILD, sarcoidosis and hypersensitivity pneumonitis rank as the top four causes of ILD-related PF, in descending order of incidence. This article explores the variation in global incidence and prevalence, genetics, diagnostic pathways, treatment approach, care gaps and recent advances in PF management. FINDINGS: The global annual incidence of ILD-related PF has more than doubled between 1990 and 2019; conservative estimates suggest current incidence of about 15-20 cases per 100 000 population. The incidence, prevalence and spectrum of ILD-related PF vary greatly across regions based on genetics, environment, industries and diagnostic rigour. Diagnosis of PF hinges on chest imaging, lung histology and expert evaluation, which remain scarce in underserved regions. About 30-60% of patients with ILD-related PF develop progressive disease over 1-2 years. Annual PF care cost averages approximately US$30 000 in developed countries. Although significant gaps remain in PF care globally, recent progress is encouraging. Key steps to enhance PF care include ensuring universal health coverage via public systems or insurance, improving access to imaging and expert diagnostic teams, incorporating telehealth technologies, accelerating drug development and expanding the availability of pulmonary rehabilitation, palliative care and lung transplantation. Emerging techniques such as gene silencing, alveolar organoids, stem cells, lung-on-a-chip and nano-based drug delivery could transform PF care in the future. CONCLUSIONS: PF is an important global healthcare challenge, especially due to its clinical heterogeneity and geographic variation. Gaps in care need to be addressed by prioritising new drug development and ensuring wider dissemination of PF-related awareness and services.