What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic Pulmonary Fibrosis (IPF) is?

The progressive scarring of the lungs that occurs when air sacs known as alveoli gradually become replaced by fibrotic tissue or scar tissue. As the scar tissue becomes thicker, it leads to stiffness in the lungs, making it difficult to breathe. Pulmonary fibrosis is found in over 200 lung disorders, so it is important for your health care provider to identify the cause of the fibrosis, because different types of fibrosis respond to different treatments.

What’s the prevalence of Pulmonary Fibrosis?

• 5,000,000 people Worldwide affected
• 200,000 patients in the United States, of these:
• 40,000 expire annually
• Typically patients are in their 40s and 50s when diagnosed, however, diagnoses have ranged
from age 7 to the 80s.

Please go to Pulmonary Fibrosis Foundation for more information at http://pulmonaryfibrosis.org/. Thank you to Pulmonary Fibrosis Foundation for ALL of their dedication and commitment to the IPF community! (ALL below information obtained from  Pulmonary Fibrosis Foundation 1.26.2017)

“The word “pulmonary” means “lung” and the word “fibrosis” means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Also, pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.”

What are the causes?

“It can be challenging to figure out what caused your pulmonary fibrosis. Sometimes doctors are able to identify one or more causes of your disease, but it is also common to end up without an answer, despite completing a large number of medical tests. Diseases where a specific cause is not identified are often called “idiopathic” – meaning the cause is not identified or the disease occurs spontaneously.

There are many forms of pulmonary fibrosis that are idiopathic. The one that many people have heard of is “idiopathic pulmonary fibrosis” or IPF, but there are many others. A few examples are:

  • Idiopathic nonspecific interstitial pneumonia (NSIP)
  • Cryptogenic organizing pneumonia (COP)
  • Sarcoidosis

When the cause of PF is known, if often falls into one of these categories:

Autoimmune diseases

Autoimmune diseases are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means “self” and “immune” refers to your immune system. With autoimmune diseases, your own immune system is attacking your lung. If you have an autoimmune disease, your immune system can cause inflammation and scarring in the lungs. Examples of autoimmune diseases that can cause PF include:

  • Rheumatoid arthritis
  • Scleroderma (now called “systemic sclerosis”)
  • Certain muscle diseases (polymyositis, dermatomyositis, and the anti-synthetase syndrome)
Occupational exposures

Pulmonary fibrosis can develop after significant exposure to a wide variety of inorganic dusts, including asbestos, silica, coal dust, beryllium, hard metal dusts.

Environmental exposures

Organic dusts, including animal proteins, bacteria, and molds, can also contribute to the development of some types of PF. Diseases caused by inhaled organic dusts are often called “hypersensitivity pneumonitis”. Exposure to radiation can also contribute to the risk of PF.

Drug Induced or Medication exposures

Some medications have been linked to the development of PF, including drugs used to treat:

  • Infections (nitrofurantoin, sulfasalazine)
  • Heart disease (amiodarone, propranolol)
  • Seizures (phenytoin)
  • Cancer (methotrexate, bleomycin, oxaliplatin, radiation therapy)
Genetic/Inherited diseases

Approximately 10-15% of those with an “idiopathic” form of PF have another family member afflicted by the disease. This is called familial pulmonary fibrosis (FPF) or familial interstitial pneumonia (FIP). A number of genes and genetic variants have been identified that are associated with the development pulmonary fibrosis, but frequently genetic tests are not performed when PF is diagnosed, as we are only just beginning to understand the significance of these genetic abnormalities. It is important to discuss the potential risks and benefits of genetic testing with a qualified genetic counselor and your medical provider. To speak with a Certified Genetic Counselor free of charge, contact Janet Talbert, MS, CGC at 800.423.8891, extension 1097.

Classification of Common Interstitial Lung Disease


The most common symptoms of PF are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.

Other common symptoms of pulmonary fibrosis include:

  • Chronic dry, hacking cough
  • Fatigue and weakness
  • Discomfort in the chest
  • Loss of appetite
  • Unexplained weight loss

How is it treated?

There is no definitive cure for Pulmonary Fibrosis but there are emerging medicines on the forefront.   Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients varies greatly.

How can I take care of myself?

If you smoke, quitting can slow the progress of the disease considerably.
You can partially control symptoms by following these guidelines for home care:

• Stop smoking.
• Avoid smoke-filled environments.
• Eat nutritious foods.
• Eat high-calorie snacks between meals if you are underweight.
• Take vitamin and mineral supplements if recommended by your doctor.
• Be as active as you comfortably can.
• Get plenty of rest and sleep.
• Learn to use relaxation techniques to reduce anxiety and fear.
• Get pneumonia immunization as well as annual flu immunizations.