Abstract

Idiopathic pulmonary fibrosis is a life-threatening, age-related lung condition with few therapy options. Healthy tissue is replaced by an altered extracellular matrix, and alveolar architecture is damaged, resulting in decreased lung compliance, disturbed gas exchange, respiratory failure, and death. Understanding of the pathophysiology and management of this condition has improved dramatically in less than a decade, and two disease-modifying medicines have been licenced globally. The presentation, pathophysiology, diagnosis, and therapy options for patients with idiopathic pulmonary fibrosis are summarized in this study. This condition has increased our understanding of lung fibrosis mechanisms, and it gives us optimism that comparable treatments can revolutionize the treatment of patients with other progressive fibrotic lung diseases. This condition was originally assumed to be caused by a persistent inflammatory process, but new data suggests that abnormally activated alveolar epithelial cells are to blame for the fibrotic response (AECs). These cells release mediators that cause fibroblast and myofibroblast foci to develop as a result of their proliferation. Excessive extracellular matrix, primarily collagens, is secreted by the fibroblast and myofibroblast foci, leading in scarring and damage of the lung architecture. The mechanisms that link idiopathic pulmonary fibrosis to ageing and inappropriate epithelial activation are unknown; however, evidence shows that improper recapitulation of developmental pathways and epigenetic alterations may play a role. Recent data on the clinical course, treatment choices, and underlying mechanisms suspected to be involved in the pathophysiology of idiopathic pulmonary fibrosis are reviewed.