Related Experiment Video
Updated: Jun 9, 2026
05:45
A Refined Aerosol-Based Intratracheal Bleomycin Delivery Method for Reproducible and Minimally Invasive Mouse Models of Pulmonary Fibrosis
Published on: January 16, 2026
Pulmonary fibrosis: evolving therapeutic pipeline.
Laura Shannon1, Sabria Islam1, Benjamin Hilary2
1Respiratory Medicine, Northern Ireland Medical and Dental Training Agency, Belfast, UK.
BMJ Medicine
|June 8, 2026
Summary
Current pulmonary fibrosis treatments slow disease progression. New agents show promise, and ongoing research aims to reverse fibrosis by targeting its underlying mechanisms.
Area of Science:
- Pulmonary Medicine
- Fibrosis Research
- Drug Development
Background:
- Current treatments for pulmonary fibrosis, including nintedanib and pirfenidone, offer limited efficacy, only slowing disease progression.
- Emerging therapies like nerandomilast have shown promise, with US approval for specific conditions, but await wider clinical adoption.
- Advancements in understanding pulmonary fibrosis pathogenesis reveal new therapeutic targets.
Purpose of the Study:
- To review current and emerging treatments for pulmonary fibrosis.
- To explore novel therapeutic targets based on advancing knowledge of disease mechanisms.
- To discuss the importance of both positive and negative trial outcomes in guiding future research.
Main Methods:
- Literature review of current treatments and emerging agents for pulmonary fibrosis.
- Analysis of pathogenic pathways involved in fibrosis, including epithelial injury and cytokine signaling.
- Evaluation of clinical trial outcomes to inform future study design.
Main Results:
- Nintedanib and pirfenidone are current global standards but do not halt or reverse fibrosis.
- Nerandomilast shows positive Phase 3 results and US approval for certain fibrotic lung diseases.
- Research is exploring multiple pathways like fibroblast activation and immune cell involvement.
Conclusions:
- Developing safer, well-tolerated treatments that target underlying fibrotic mechanisms is crucial.
- Insights from both successful and unsuccessful clinical trials are vital for optimizing future therapeutic strategies.
- The ultimate goal is to move beyond slowing fibrosis to achieving disease reversal.
Related Concept Videos
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
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...
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...
01:20COPD: Pathogenesis and Clinical Features
Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
01:30Pulmonary Hypertension: Classification and Pathogenesis
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
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...
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...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...
01:29Chronic Obstructive Pulmonary Disease-V: Management
Managing Chronic Obstructive Pulmonary Disease (COPD) involves a multifaceted approach to reduce symptoms, prevent exacerbations, improve overall health status, and slow disease progression. Key strategies include lifestyle modifications, pharmacotherapy, supportive therapies, and, in some cases, surgery. Here is an overview of the primary COPD management strategies:
Smoking Cessation
Smoking Cessation