Tweaking the Complex Fibrogenic Role of Lymphocytes in Idiopathic Pulmonary Fibrosis.

Abstract

Idiopathic pulmonary fibrosis is a deadly lung disease primarily affecting aged individuals. Even though there are two U.S. Food and Drug Administration-approved drugs, nintedanib and pirfenidone, with a recent addition of another drug, nerandomilast, yet they only reduce the progress of the disease. The mean survival rate is between 5 and 7 years even after treatment with antifibrotics. Cells of lymphoid lineage have been long reported to modulate the outcome of pulmonary fibrosis. In this review, we discuss how the cell of lymphoid lineage regulates the inflammatory niche within the lungs, leading to the development and progress of pulmonary fibrosis. The review also addresses possible therapeutic strategies that can be leveraged by specifically targeting the lymphoid cells in the pulmonary fibrotic niche.