Idiopathic Pulmonary Fibrosis

Pulmonary fibrosis (PF) is a disease of the respiratory system caused by the thickening and stiffening of lung tissue that leads to the formation of scar tissue. When physicians are unable to identify the root cause of the disease, it is called idiopathic pulmonary fibrosis, or IPF (idiopathic means “of unknown cause”). PF can often be the result of factors and injuries that cannot be definitively identified.

As with PF, no single test can effectively diagnose IPF and a battery of diagnostic tests is often required. IPF is what is called an interstitial lung disease — or a disease that primarily affects the interstitium (the tissue and space around the air sacs of the lungs), rather than the airways or blood vessels. There are many types of interstital lung disease that lead to inflammation and/or fibrosis. Because treatments vary among this group of lung diseases, it is important that IPF be correctly diagnosed before the initiation of a treatment.

Common risk factors for IPF

Known risk factors for IPF are:

  • Genetics. Up to 20 percent of IPF patients have another family member with an interstitial lung disease.
  • Cigarette smoking. About 75 percent of IPF patients are current or former cigarette smokers
  • Acid reflux. About 75 percent of IPF patients have symptoms of acid reflux/gastroesophageal reflux disease (GERD)
  • Male gender. About 75 percent of IPF patients are males.
  • Older age. Most IPF patients are age 50 or older.

Symptoms of IPF

Breathlessness (or shortness of breath): This symptom usually first appears during exercise. Breathlessness can affect daily activities like showering, climbing stairs, getting dressed and even eating.  As scarring in the lung tissue worsens, breathlessness progresses.

Chronic cough: About 85 percent of IPF patients suffer from a chronic cough, or a cough that lasts longer than eight weeks. IPF-associated cough is often dry, but people may also cough up sputum or phlegm.

Other symptoms can include chest pain or tightness, unexplained weight loss, loss of appetite, loss of energy, aching in the muscles and joints, and “clubbing” of the digits (a wider and rounder look to the shape of the fingers and, sometimes, the toes).

Speak with your doctor

  • Prepare a written list of questions you want answered, and take your time. Make the most important questions your priority. Take notes when the doctor is answering – you may think you’ll remember but a memory aid often helps. If you don’t understand what the doctor is explaining to you, say so and ask for details.
  • Be honest about your symptoms — always. Bring along all your medications, including herbal remedies. If you’re not sure if you’re taking your medicines correctly, your doctor can help you.
  • Bring a friend or a family member with you, that person can help you remember details and give you support.
  • Ask your doctor about support groups and programs in your area for patients with IPF and other lung diseases.
  • Ask about joining a pulmonary rehabilitation program.
  • Don’t forget to follow up, and update your doctor on the progression of your disease. Such updates will help to ensure you are given the best treatment plan possible.

Available treatments

Although no cure exists for IPF,  the U.S. Food and Drug Administration (FDA) has two approved drugs to treat IPF: Esbriet (pirfenidone) and Ofev (nintedanib).

Pulmonary rehabilitation programs can also assist people with lung diseases. These programs range from breathing strategies to exercise to counseling, and help in teaching patients  how to breathe more effectively. They also teach patients how to go about daily activities with fewer episodes of breathlessness. They complement, but do not necessarily replace, medical therapy.

In more severe cases, a lung transplant might be necessary. It is very important to discuss with your doctor if lung transplant is right for you. If you qualify, you will be placed on a waiting list for organ donation, which can be very long. If you do get a transplant, you may need to take immune-suppressant medication for the rest of your life.

Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.