Related Experiment Video
Updated: Mar 22, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Pharmacological management of IPF
Raphael Borie1,2,3, Aurelien Justet1,2,3, Guillaume Beltramo1,2,3
1APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a deadly disease with a median survival of approximately three years in historical cohorts. Despite increased knowledge of disease pathophysiology and selection of more targeted therapy, main clinical trials yielded negative results. However, two agents, pirfenidone and nintedanib, were recently shown to be effective in IPF and received marketing authorization worldwide. Both drugs significantly reduce functional decline and disease progression with an acceptable safety profile. Yet, none of these drugs actually improves or even stabilizes the disease or the symptoms perceived by the patient. Several other treatments and combinations are currently tested, and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.
Related Concept Videos
Chronic Obstructive Pulmonary Disease-V: Management
Smoking Cessation
COPD: Management Using Bronchodilators and Corticosteroids
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Inflammatory Bowel Disease IV: Pharmacological Management
Pharmacologic...
Cystic Fibrosis: Management
Sinus disease and chronic...

