Progress in the Battle Against Pulmonary Fibrosis

Take a deep breath and exhale. You breathe life-giving oxygen 20,000 times every day without even thinking about it.

However, for people living with pulmonary fibrosis, every breath can be a struggle.

“Pulmonary” means lungs and “fibrosis” means scar tissue. Pulmonary fibrosis (PF) refers to a group of over 200 different diseases that involve scarring of the lungs. It occurs when scar tissue builds up in the walls of the lung’s air sacs, making it difficult for oxygen to get into the blood. The disease typically gets worse over time, but the rate at which it progresses can vary from patient to patient. Sometimes there is a known cause for PF, but in many cases it’s not possible to determine why someone develops pulmonary fibrosis.

“Even after extensive medical testing, patients are commonly diagnosed with idiopathic pulmonary fibrosis, which means we can’t identify the cause,” said Ross Summer, MD, a pulmonologist at the Jane and Leonard Korman Respiratory Institute — Jefferson Health and National Jewish Health. “Regardless of the cause, early and accurate detection is essential to successfully managing the disease.”

Every year, up to 50,000 Americans learn that they have idiopathic PF.

PF Causes and Symptoms

While diagnosis isn’t necessarily easy, sometimes it’s possible to pinpoint a cause. The common causes include:

  • Autoimmune diseases such as rheumatoid arthritis and systemic scleroderma
  • Exposure to dust and chemicals
  • Exposure to mold and bacteria
  • Medications to treat seizures, cancer, heart disease, and infections

In up to 15 percent of idiopathic pulmonary fibrosis, the patient has a family member who also has PF. This suggests that there may be a genetic abnormality responsible for the disease in some cases.

“Some of the causes of PF are reversible, while others are not,” said Dr. Summer. “Treatment ranges from observation and symptom management to use of medications designed to slow the progression of the disease.”

The hallmark symptoms of pulmonary fibrosis are shortness of breath and coughing. In the early stages, patients may find it’s difficult to catch their breath when exercising. However, as the disease gets worse, even normal daily activities will make it difficult to breathe.

While problems other than PF can also make it difficult to breathe, this symptom is a sure sign that it’s time for a checkup with your doctor. The earlier the problem is detected, the better your chances are of successfully treating the disease.

How Pulmonary Fibrosis Treatment is Advancing

Previous treatment for pulmonary fibrosis focused on immunosuppressant drugs, which block signals from immune cells that lead to scarring. The problem with this approach is that these drugs suppress the patient’s entire immune system, making them more vulnerable to infections and other illnesses.

Other researchers have been focusing on therapies that inhibit scarring in the lungs. While this approach works, it does nothing to fix the existing scarring in the lungs that impedes the efficient exchange of oxygen and other gasses.

Dr. Summer and the pulmonary fibrosis research team at Jefferson are working on a novel approach. They discovered significant differences in the way young cells in the lungs repair DNA compared to older cells that introduce mutations when they attempt to fix damaged DNA. It’s this DNA damage that leads to scarring in pulmonary fibrosis.

“The team will apply this discovery to the lung’s stem cells, which repair damaged tissue,” said Dr. Summer. “By creating drugs that target these cells, we hope to be able to slow the spread of fibrosis in the lungs.”

While additional research and clinical trials will still need to take place, Dr. Summer and his team are providing new hope for better treatment options for patients with pulmonary fibrosis.

 

 

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