Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease characterised by the irreversible replacement of normal lung parenchyma with stiff scar tissue. The prognosis of IPF is very poor, with a median survival worse than many cancers at 2–3 years, and this condition is associated with increasing incidence and mortality rates worldwide [1–4]. Despite a dramatic increase in IPF clinical trials over the past 20 years, there remain only two therapies approved for use in IPF: nintedanib and pirfenidone [5, 6]. These antifibrotic drugs are not curative, do not improve patient symptoms or functional status and only slow down loss of lung function. Therefore, work to understand the key cells and molecular pathways that drive IPF is essential to identify new therapies for this devastating disease.
Goodwin, A. T., Noble, P. W., & Tatler, A. L. (2022). Plasma cells: a feasible therapeutic target in pulmonary fibrosis?. European Respiratory Journal, 60(5), Article 2201748. https://doi.org/10.1183/13993003.01748-2022