An estimated 13-20 out of every 100,000 people around the globe have idiopathic pulmonary fibrosis (IPF). The condition often has a poor prognosis (3-5 years following diagnosis) and there are few effective treatment options. While patients may have the option to use medication, try oxygen therapy, or even receive a lung transplant, the fibrosis (scarring) caused by IPF cannot be reversed. However, according to News Medical, new research has discovered a new molecular target which could be used to develop more effective treatment options for patients. See the full findings in the Journal of Experimental Medicine.

The Research

In patients with IPF, researchers believe an excessive amount of collagen is deposited by lung cells into the other tissue. As this happens, the tissue begins to tighten. Additionally, this prompts the activation of myofibroblasts, which create even more collagen. This cycle causes the lung tissue to continue hardening, stiffening, and scarring. Thus, some researchers believe that it is important to find a way to regenerate lung tissue and stop the production of excess collagen. However, nobody is entirely sure what mechanisms or processes are needed to perform this.

For this study, Yong Zhou, Jing Qu, and a team of researchers explored these mechanisms in mice models of IPF. They found that myofibroblasts sourced from patients with IPF, and mice models, all had high levels of MDM4, a type of protein. More so, they found that MDM4 is produced by the body as a response to fibrosis. Next, researchers worked to lower MDM4 levels. As a result, myofibroblasts had difficulty surviving and could be more easily removed from fibrotic areas. Even more interestingly, taking the MDM4 gene out of fibroblasts and myofibroblasts helped mice reduce lung fibrosis and achieve some semblance of remission.

Later, researchers treated the mice models of IPF with chebulic acid. They found that doing so helped to soften areas of fibrosis and lower MDM4. Altogether, this suggests that addressing MDM4 and finding ways to inhibit or reduce it could resolve the lung fibrosis which characterizes IPF.

Idiopathic Pulmonary Fibrosis (IPF)

The aptly named idiopathic pulmonary fibrosis (IPF) can be broken down into 3 concepts: idiopathic, or of an unknown cause; pulmonary, or relating to the lungs; and fibrosis, or the hardening, scarring, and thickening of tissue. So IPF occurs when the lung tissue begins scarring but with no known cause. As the fibrosis worsens, oxygen is unable to enter the bloodstream, causing health issues. Despite the fact that nobody knows the cause, many believe it could be due to a mix of genetic and environmental factors. Those older than 70 are also more likely to develop the condition. IPF can lead to other conditions, such as lung cancer. Symptoms include:

• Shortness of breath and/or difficulty breathing
• A persistent dry, hacking cough
• Pneumonia
• Pulmonary blood clots
• Gastroesophageal reflux
• Appetite loss
• Unintended weight loss
• Clubbed fingers and toes
• Fatigue
• Chest pain