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Challenges in the Understanding, Diagnosis, and Treatment of Idiopathic Pulmonary Fibrosis (IPF)

Eickelberg O.

Respiratory Drug Delivery 2020. Volume 1, 2020: 1-8.

Abstract:

Interstitial lung diseases (ILD) represent a large group of heterogeneous and fibrosing lung diseases, with idiopathic pulmonary fibrosis (IPF) as the most common and severe form. Patients with lung fibrosis exhibit alterations to the interstitium of the lung, distorted pulmonary architecture, and a significantly reduced gas exchange ability leading to respiratory insufficiency. Lung fibrosis can develop due to gene/patient–environment interactions, distorted immune functions, or in the setting of certain genetic scenarios. While progressive lung fibrosis is fatal, some patients remain stable for prolonged periods. To date, we lack robust molecular biomarkers that predict the susceptibility, onset, severity, or progression of lung fibrosis. Further, while two FDA-approved therapies have been shown to slow down progression of lung fibrosis, no therapy is currently available that reverses or cures IPF. Novel models of lung fibrosis have recently been developed that will aid in furthering our understanding and therapeutic development in ILD.