Pulmonary fibrosis has a 5-year survival and demands new treatments to help patients live longer and better.
Pulmonary fibrosis is a rare disease that appears slowly, forming scars and damaging lung tissue to the point that breathing becomes difficult. With a chronic and progressive course, this pathology usually leads to death within 3 to 5 years and has few treatments so far. But a clinical trial in Latin America aims to investigate a new drug that has an innovative mechanism against different presentations of pulmonary fibrosis.
The so-called “interstitial lung diseases” include different types of fibrosis and inflammation. Pulmonary fibrosis can occur without apparent cause and is therefore called “idiopathic fibrosis”. This severe fibrosis has a prevalence of about 1 to 16 cases per 100,000 inhabitants, depending on the country. In Argentina, there are between 6,000 and 12,000 cases of idiopathic pulmonary fibrosis, according to the Argentine Association of Respiratory Medicine.
But fibrosis can also be the consequence of a disease -such as tuberculosis, scleroderma or rheumatoid arthritis-; the result of an environmental aggression (such as cigarette smoke or asbestos); or even the result of chemotherapy against cancer.
Learn about the new treatment being studied for pulmonary fibrosis hereThe challenge of diagnosing pulmonary fibrosis
In Latin America, most patients take two years to receive a correct diagnosis, which is obtained by auscultation of the lungs (where velcro-like crackling sounds are heard) and computed tomography.
Pulmonary fibrosis is often confused with interstitial pneumonias, which affect the small space between the walls of the alveoli and the blood vessels. Inflammatory cells accumulate in this interstitium, causing shortness of breath and coughing. Over time, these pneumonias lead to hardening of the lung tissue and remodeling of the lung, which resembles the structure of a honeycomb.
In most cases, fibrosis – the hardening and scarring of lung tissue – appears after the age of 40 and is recognized by the following symptoms:
- Extreme fatigue
- Shortness of breath
- Choking sensation on exertion
- Dry cough
- Heartburn
Pulmonary fibrosis affects an estimated 700,000 people worldwide, impacting twice as many men as women. In fact, in the United States alone, approximately 40,000 cases are reported, with a higher prevalence among Black individuals compared to Hispanics and whites.
The disease begins when lung tissue is damaged — often by a virus or toxic substance — and the body attempts to repair it through scarring. However, the problem arises when the scar doesn’t fade as it should. Instead, collagen and fibronectin accumulate over time, leading to persistent scarring. As a result, the cells that cover the lungs (fibroblasts) start to multiply and transform, ultimately altering the lung’s entire structure. This remodeling severely impacts the exchange of oxygen and carbon dioxide in the pulmonary alveoli.
As fibrosis advances, specialists may prescribe oxygen therapy to help patients breathe and maintain their daily activities. Yet, over time, the lungs become so stiff that they can no longer effectively expel carbon dioxide or take in enough oxygen. Eventually, breathing becomes extremely difficult, making even simple tasks impossible.
How to increase survival from pulmonary fibrosis
Although the process was considered irreversible until recently – and could only be counteracted by organ transplantation – there are now a couple of new drugs (pirfenidone and nintedanib) that can partially delay the progression of fibrosis. In addition, new molecules capable of halting respiratory deterioration in the long term and with fewer adverse effects are being experimented with.
One of these innovative molecules, developed by the Bristol Myers Squibb laboratory, is being tested in several countries around the world, including the United States, Argentina, Brazil, Chile, Mexico, Colombia and Peru. Patients with idiopathic or progressive pulmonary fibrosis can voluntarily participate in the clinical trial.
Bibliographic references
Henderson, N. C., Rieder, F., & Wynn, T. A. (2020). Fibrosis: from mechanisms to medicines. Nature, 587(7835), 555–566. https://doi.org/10.1038/s41586-020-2938-9
Nishioka, Y., Araya, J., Tanaka, Y., & Kumanogoh, A. (2024). Pathological mechanisms and novel drug targets in fibrotic interstitial lung disease. Inflammation and regeneration, 44(1), 34. https://doi.org/10.1186/s41232-024-00345-2
