What is Pulmonary Fibrosis

About the Information Provided in This Section

Patients and family members often call the Pulmonary Fibrosis Foundation with the news they have just been diagnosed with Idiopathic Pulmonary Fibrosis, or IPF, oftentimes feeling frightened, confused and concerned. Most patients find themselves totally frustrated by the lack of available information on IPF. Physicians often don’t have the necessary time to explain to their patients all the details of the disease or help the patients deal with the trauma of being told that he/she has an illness for which there is no cure.

The Pulmonary Fibrosis Foundation is deeply aware of these concerns – most of our staff are patients or caregivers themselves, and they work with patients every day to assist them in understanding their disease and empowering them to learn more about IPF.

It is important to note that there is no consistent standard of care for IPF in the medical community, and disease progression varies greatly in patients – your physician may have discussed this challenge with you. Because of these issues, it is critically important for patients to understand their condition, and ask important questions of their physician to ensure they are being treated as aggressively as possible based on their individual symptoms. This section of our Web page is intended to help patients achieve this goal.

Secondly, the terminology concerning these diseases is often confusing to patients. IPF is a specific disease within a classification of diseases referred to as interstitial lung diseases (ILD). IPF implies that there is no obvious or discernible etiology, thus it is called “idiopathic”. If there is a clear association with another disease such as scleroderma or rheumatoid arthritis, or a side affect resulting from of drug an individual may have been prescribed, then the cause of the disease is no longer considered idiopathic. In such situations the disease would be referred to as pulmonary fibrosis secondary to scleroderma, or secondary to rheumatoid arthritis. As we learn more about the genetic and peripheral biomarkers of IPF, new, more precise terminology may develop.

Lastly, please remember that this information is a brief overview of IPF and is for educational purposes only. It is not intended to be a substitute for professional medical advice. Always consult your personal physician or healthcare provider with any questions you may have regarding your specific medical condition.

And please know that we are here to help you. You can contact the Pulmonary Fibrosis Foundation with any questions or concerns you have about IPF during the course of your care.