Pulmonary fibrosis involves scarring in the lungs so that lung tissue thickens and stiffens, making it difficult for the body to take in needed oxygen. It is an interstitial lung disease of often-unknown origin. A hacking cough, shortness of breath, and fatigue are common symptoms. While there is no cure, various treatments help alleviate symptoms of pulmonary fibrosis.
Pulmonary fibrosis involves scarring in the lungs. When tissue deep within the lungs develops scarring, and becomes thick and stiff, the body is unable to take in air properly. “Fibrosis” is scarring caused by excess fibrous connective tissue. In pulmonary fibrosis, scarring progressively decreases the body’s ability to absorb oxygen.<
Pulmonary fibrosis is considered an interstitial lung disease, and many cases are ascribed to exposure to substances such as asbestos. Additionally, some believe it arises due to use of certain medications. However, many believe numerous cases are misdiagnosed as other interstitial lung diseases, when, in fact, they are cases of idiopathic pulmonary fibrosis. The Coalition for Pulmonary Fibrosis states, “There is no known cause, no FDA approved treatments and no cure for IPF. IPF is one of the few remaining diseases in which this is the case.”
However, smoking and exposure to certain dusts (metal and wood) are possible risk factors. There likely is a genetic component involved in the risk of contracting the disease. Other possible risk factors may include some viral infections and exposure to bacteria and animal proteins. Additionally, more men have pulmonary fibrosis than women do.
The symptoms of pulmonary fibrosis include a dry, constant cough that goes on for more than 30 days, shortness of breath and shallow breathing, fatigue, joint and muscle pain, and unintended weight loss. Additionally, physicians using a stethoscope can hear a crackling sound in the lungs of pulmonary fibrosis patients. Often, symptoms progress very slowly, but not always.
The cause of pulmonary fibrosis often is unknown. In such cases, the disease is called idiopathic pulmonary fibrosis. An underlying connective tissue disease causes some cases of pulmonary fibrosis. Two examples are rheumatoid arthritis and lupus, both autoimmune disorders.
At present, there is no way to remove the scarring present in pulmonary fibrosis. Additionally, there are no medications proven to slow or halt the progression of pulmonary fibrosis. However, many physicians use a drug regimen to treat pulmonary fibrosis. The “cocktail” includes anti-inflammatories or steroids, immune-suppressing drugs, and antioxidants to treat the disease. However, recent evidence indicates that those using such therapy must be closely monitored.
Other treatments for pulmonary fibrosis involve addressing symptoms. Codeine based cough medicines may reduce coughing, and bone-strengthening medications such as Vitamin D and calcium may help prevent bone loss. Oxygen therapy may help when blood gas levels sink too low. Pulmonary therapy, consisting of various activities and treatment to improve the patient’s overall well-being, is a standard practice. In some cases, a lung transplant is warranted.
Research continues to find new pulmonary fibrosis treatments. Some of the research on pulmonary fibrosis concentrates on creating and/or identifying medications specifically useful for decreasing the inflammation associated with disease. Researchers also seek treatments for preventing and/or reducing any of the lung scarring caused by idiopathic pulmonary fibrosis.
The information on this page is not intended to be a substitute for professional medical advice, diagnosis, or treatment. For more information about pulmonary fibrosis, talk to your doctor or primary care provider.
Page last updated: October 14, 2018