CONDITIONS

What Is Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis, or IPF, is a chronic, progressive—and generally fatal—lung disease, characterized by scarring that thickens the lining of the lungs. This causes irreversible loss of the tissue’s ability to transport oxygen to the rest of the body, ultimately robbing a patient of the ability to breathe. At present, there is no known cause, no FDA-approved treatments, and no cure for IPF.

About 128,000 people in the Unites States, with about 48,000 new cases diagnosed per year. IPF usually affects patients 50 to 70 years of age, and occurs more often in men than women—with a male to female ration of 2:1. Men tend to be diagnosed at a later stage in the disease.

There are some activities, medical conditions, and medications that have been linked to an increased risk of developing idiopathic pulmonary fibrosis or IPF. These include: smoking, certain viral infections (like Epstein-Barr, hepatitis C, HIV, and more), certain medicines, radiation, environmental toxins, genetics, and GERD.

The GERD connection is large—affecting 90% of those with IPF. Researchers have found that 9 out of 10 people who have IPF also have gastroesophageal reflux disease or GERD – a condition in which acid from your stomach backs up into your esophagus and throat. It is thought that some of those who have GERD may regularly breathe in tiny droplets of acid from their stomachs, which can, in turn, injure their lungs and lead to IPF. More research is being done to explore the date behind this theory.

To see a slideshow about what you need to know about Idiopathic pulmonary fibrosis (IPF)

What Causes Idiopathic Pulmonary Fibrosis (IPF)

“Idiopathic” means that the cause is not known—and thus far, the cause of IPF is exactly that, unknown. According to researchers, IPF creates an out-sized, uncontrolled inflammatory response that produces scar tissue. It is unknown what initiates this cycle. Over time, this scar tissue surrounds the thin-walled air sacs in the lungs, making the tissue thicker and stiffer. Breathing becomes more difficult, and the lungs gradually lose their ability to oxygenate the rest of the body.

Since the signs and symptoms of IPF develop over time, and may not begin to appear until the disease has done serious damage to your lungs. Once symptoms do occur, they will likely worsen over time. The most common signs and symptoms are shortness of breath and a dry hacking cough that doesn’t get better. Shortness of breath is typically the main symptom of IPF. At first, you may be short of breath only during exercise, but eventually you’ll likely feel breathless even at rest. The cough also gets worse with time, and ultimately, someone with IPF will have repeated bouts of coughing that cannot be controlled.

 

Risk Factors For Idiopathic Pulmonary Fibrosis (IPF)

There are some activities, medical conditions, and medications that have been found to be linked to an increased risk of developing idiopathic pulmonary fibrosis or IPF. These include:

  • Smoking
  • GERD
  • Certain viral infections (like Epstein-Barr, hepatitis C, HIV, and more)
  • Certain medicines like nitrofurantoin, sulfasalazine, amiodarone, propranolol, methrotrexate, bleomycin radiation,
  • Environmental toxins to dusts containing silica, bacteria, and animal proteins or to gases and fumes
  • Family history of IPF

The link between GERD and IPF is particularly strong. Researchers have found that 9 out of 10 people who have IPF also have gastroesophageal reflux disease or GERD — a condition in which acid from your stomach backs up into your esophagus and throat. It is thought that some of those who have GERDmay regularly breathe in tiny droplets of acid from their stomachs, which can, in turn, injure their lungs and lead to IPF. More research is being done to explore the date behind this theory.

Diagnosing Idiopathic Pulmonary Fibrosis (IPF)

The doctor will ask about your symptoms and medical history. A physical exam will be done. One or more of the following tests may be performed:

  • Chest x-ray or CT scan to view the lungs and check for scarring
  • Pulmonary function tests  to measure the size and effectiveness of the lungs
  • Blood test to tell how well the lungs are taking up oxygen
  • Exercise test on treadmill or stationary bicycle to measure how well the lungs and heart work during physical activity
  • Bronchoalveolar lavage fluid is put into the airways and then removed to study the cells and check for signs of inflammation
  • Lung biopsy a small sample of lung tissue is removed and studied; usually required to confirm a diagnosis of IPF

Idiopathic pulmonary fibrosis causes the same kind of scarring and symptoms as some other lung diseases, making it hard to diagnose. It’s important to seek medical help from a lung specialist as soon as you have symptoms.

Your doctor will diagnose IPF based on your medical history, a physical exam, and test results. Tests will help rule out other possible causes for your symptoms, and can show where and how badly your lungs are damaged.

 

Symptoms of Idiopathic Pulmonary Fibrosis (IPF)

The signs and symptoms of IPF develop over time, and may not begin to appear until the disease has done serious damage to your lungs. Once symptoms do occur, they will likely worsen over time. The most common signs and symptoms are shortness of breath and a dry hacking cough that doesn’t get better. Shortness of breath is typically the main symptom of IPF. At first, you may be short of breath only during exercise, but eventually you’ll likely feel breathless even at rest. The cough also gets worse with time, and ultimately, someone with IPF will have repeated bouts of coughing that cannot be controlled.

Symptoms of IPF tend to worsen over time and may include:

  • Rapid, shallow breathing
  • Gradual, unintended weight loss
  • Fatigue or sense of malaise
  • Achy muscles and joints
  • Clubbing, which is the rounding and widening of the fingers and toes

Prognosis

While research is being done into IPF, at present there is no effective treatment that slows disease progression. About two-thirds of IPF patients pass away within five years.

Living With Idiopathic Pulmonary Fibrosis (IPF)

While there is no cure yet for IPF, there are 5 things you can do that can help you to feel and stay better!

1.  If you’re still smoking, QUIT! It’s the most important thing you can do.
2.  Get vaccinated against influenza and pneumococcus, which protects against infections that can be particularly severe if you have lung disease.
3.  Stay active. Physical activity can help with both your physical and mental health, maintain your strength and lung function, and reduce stress.
4.  Follow a healthy diet. A healthy diet includes a variety of fruits and vegetables
5.  Eat smaller, more frequent meals, because eating this way may relieve stomach fullness, which can make it harder to breathe.

 

Screening

There is no regular screening for IPF; rather, if symptoms emerge, diagnostic tests are then done.

Prevention

While there is no proven way to prevent IPF, avoiding smoking may help.

Medication And Treatment

While doctors may prescribe medications, oxygen therapy, pulmonary rehabilitation, and lung transplant to treat idiopathic pulmonary fibrosis; at present, there is no cure. The aim of treatment is to suppress symptoms to the best degree possible.

A healthy lifestyle may also help slow the disease and includes:

  • Healthy diet
  • Regular exercise
  • Rest
  • Not smoking

Medication is the main form of treatment for IPF, and is used to manage symptoms. It does not work for some people. Options may include:

  • Combination of prednisone to reduce inflammation and cytoxan or azathioprine to reduce the body’s immune response has been a typical course of treatment, but views on this approach are changing
  • To dampen the progression of the fibrosis your doctor may recommend:
    • High dose n-acetylcysteine
    • Pirfenidone
    • Interferon-gamma 1b
    • Coumadin
    • Etanercept
    • Bosentan
    • Imatinib
    • Sildenafil
    • Pirfenidone
    • Colchicine
    • Methotrexate
    • Penicillamine
    • Cyclosporine
  • Some people may need to receive supplementary oxygen, which will help them breathe.
  • A pulmonary rehab program may also improve lung function.
  • Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment.

 

Complementary and Alternative Treatment

At present there is no good medical treatment for pulmonary fibrosis. Lung transplantation is a last resort, there are few alternative treatments, however, that have shown some promised in studies.

Acetylcysteine is a nutrient being studied, which may slow disease progression. A randomized, double-blind, placebo-controlled, multicenter study was conducted on the use of acetylcysteine that sought to assess the effectiveness of a high oral dose (600 mg three times daily) added to standard therapy with prednisone plus azathioprine, over the course of a year. The study concluded that therapy with acetylcysteine, at a dose of 600 mg three times daily, added to prednisone and azathioprine, preserves vital lung capacity in those with IPF better than standard therapy does alone

Proathocyanidins are also being studied.

When To Contact A Doctor

The main symptoms of IPF are shared with many other pulmonary diseases, therefore, it’s a good idea to contact a doctor if you experience the following:

  • Rapid, shallow breathing
  • Gradual, unintended weight loss
  • Fatigue or sense of malaise
  • Achy muscles and joints

 

 

Questions For Your Doctor

The Pulmonary Fibrosis Foundation can help you find an IPF specialist near you.

Questions For A Doctor

You may want to ask your doctor the following questions:

  • How far progressed is my disease?
  • How much scarring has occurred in my lungs?
  • What can I expect?
  • What can I do to best support my health?
  • What course of treatment is right for me?
  • Can I exercise with IPF?
  • What dietary changes should I make?
  • Do I need oxygen therapy?
  • Am I a candidate for a lung transplant?