What causes pulmonary fibrosis?

You may never have heard of pulmonary fibrosis (PF) or interstitial lung disease (ILD).  Interstitial lung diseases are relatively rare compared to chronic pulmonary disease (COPD), a much more common breathing-related ailment. 

Pulmonary fibrosis may be caused by exposure to harmful chemicals or environmental irritants or by other diseases such as rheumatoid arthritis. For some patients, the cause cannot be determined. In this case, the diagnosis is called idiopathic.

Pulmonary fibrosis (PF) is a condition that isn’t just caused by just one disease. More than 200 different lung diseases, types of Interstitial Lung Disease (ILD), all can cause inflammation or scarring of the lungs. The scarring makes it harder for the lungs to extract oxygen and transfer it to the other organs in your body.

Available Therapies Can Help

While there is no cure available for PF or ILD currently, medications and treatment are available that can significantly slow the progression of lung fibrosis. The US Food and Drug Administration has approved two drugs to treat pulmonary fibrosis. And other therapies such as pulmonary rehabilitation and other medicines to manage symptoms can slow the disease progression, maintain your overall health, and improve your quality of life.

While you may have heard or read that the average life span for patients is 2 to 3 years after diagnosis, that prediction is based on old data and is not true for many of the fibrotic lung diseases. Many patients live much longer and not all diseases progress. Patients’ disease may progress rapidly or slowly. Medical research and clinical trials may offer more effective treatments soon.

A lung transplant is an option for some pulmonary fibrosis patients who qualify. A lung transplant is a major operation with risks and must be followed by long-term medications. And, there are not enough donor lungs for all patients. For those who are able to have it, it is a life-changing option.