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Pulmonary Fibrosis

What is pulmonary fibrosis?

Highlights

  1. Pulmonary fibrosis is a condition that causes lung scarring and stiffness.
  2. The cause is often unknown.
  3. No cure is available.

Pulmonary fibrosis is a condition that causes lung scarring and stiffness. This makes it difficult to breathe. It can prevent your body from getting enough oxygen and eventually lead to respiratory failure, heart failure, or other complications.

Autoimmune diseases, exposure to certain chemicals, and genetics can raise your risk of developing pulmonary fibrosis. But in many cases, the exact cause of the condition is unknown.

What are the symptoms of pulmonary fibrosis?

Symptoms

You can have pulmonary fibrosis for some time without any symptoms. Shortness of breath is typically the first symptom that develops. Other symptoms can include:

  • a chronic, dry, and hacking cough
  • weakness
  • fatigue
  • curving of the fingernails, which is called clubbing
  • weight loss
  • a rapid heart rate

Because the condition generally affects older adults, early symptoms are often misattributed to age or a lack of exercise. Your symptoms may seem minor at first and progress over time. But they can vary from one person to another. Some people with pulmonary fibrosis become ill very quickly.

What causes pulmonary fibrosis?

Causes

The causes of pulmonary fibrosis can be divided into four categories:

  • autoimmune diseases
  • chemical exposure
  • unknown causes
  • genetics

Autoimmune diseases

Autoimmune diseases cause your body’s immune system to attack itself. Autoimmune conditions that can lead to pulmonary fibrosis include:

  • rheumatoid arthritis
  • lupus erythematosus, which is commonly known as lupus
  • scleroderma
  • Churg-Strauss syndrome
  • polymyositis
  • dermatomyositis
  • polyangiitis

Chemical exposure

Exposure to chemicals can also contribute to pulmonary fibrosis. For example, cigarette smoke contains many chemicals that can damage your lungs and lead to this condition. Other things that can damage your lungs include:

  • asbestos fibers
  • grain dust
  • silica dust
  • certain gases
  • radiation

Some medications may also raise your risk of developing pulmonary fibrosis. If you have a family history of the condition, you should speak with your doctor before taking medications that can damage your lungs, including certain:

Unknown causes

In many cases, the cause of pulmonary fibrosis in unknown. When this is the case, the condition is called idiopathic pulmonary fibrosis (IPF). According to the National Heart, Lung, and Blood Institute, most people with pulmonary fibrosis have IPF.

Genetics

About 10 to 15 percent of people with IPF have another family member with pulmonary fibrosis, reports the Pulmonary Fibrosis Foundation. In these cases, it’s known as familial pulmonary fibrosis or familial interstitial pneumonia. Researchers have linked some genes to the condition, but more research about what role genetics plays is necessary.

Who is at risk of pulmonary fibrosis?

Risk Factors

You’re more likely to be diagnosed with pulmonary fibrosis if you:

  • are male
  • are between the ages of 40 and 70
  • have a history of smoking
  • have a family history of the condition
  • have an autoimmune disorder associated with the condition
  • have taken certain medications associated with the disease
  • have undergone cancer treatments, particularly chest radiation
  • work in an occupation associated with increased risk, such as mining, farming, or construction

How is pulmonary fibrosis diagnosed?

Diagnosis

Pulmonary fibrosis is one of more than 200 types of lung diseases that exist. Because so many different lung diseases exist, your doctor may have difficulty identifying that IPF is the cause of your symptoms. In a survey by the Pulmonary Fibrosis Foundation, 55 percent of respondents reported being misdiagnosed at some point. The most common misdiagnoses were asthma, pneumonia, and bronchitis.

The most definitive way to diagnose pulmonary fibrosis is by taking a tissue sample. Your doctor can take a tissue sample using bronchoscopy. In this procedure, they’ll pass a small tube through your mouth to your lungs. Then, they’ll pass a small brush, forceps, or a needle through the tube to collect a small sample of tissue.

If they need a larger tissue sample, your doctor might perform a surgical biopsy. They’ll make an incision in your chest to access your lung tissue and collect a sample. They can examine the biopsied tissue to check for signs of pulmonary fibrosis and determine how far your condition has progressed.

Your doctor may also use a variety of other tools to diagnose pulmonary fibrosis or rule out other conditions. These may include:

  • imaging scans, such as a chest X-ray, CT scan, or echocardiogram
  • blood tests to assess the oxygen levels in your blood
  • a sputum sample to check for signs of infection
  • a spirometry test to measure your lung capacity
  • a treadmill stress test to learn how your lungs respond to physical activity

How is pulmonary fibrosis treated?

Treatment

No cure is available for pulmonary fibrosis. Your doctor can’t reverse the lung scarring, but they can prescribe treatments to help improve your breathing and slow the progression of the disease.

For example, your doctor may prescribe one or more medications, such as:

  • prednisone to suppress your immune system and reduce inflammation
  • methotrexate or cyclosporine to suppress your immune system
  • N-acetylcysteine, which is an antioxidant that doctors often prescribe along with other drugs to help slow the progression of the disease
  • pirfenidone, which is an antifibrotic drug that can help reduce inflammation and scarring in the lungs

Your doctor may also recommend oxygen therapy to help you breathe and sleep more easily. They’ll ask you to wear a plastic tube in each nostril to receive oxygen from a tank.

They may also recommend pulmonary rehabilitation. This treatment involves a program of exercise, education, and support to help you learn how to breathe more easily.

Your doctor may also encourage you to make changes to your lifestyle. These changes may include the following:

  • You should avoid secondhand smoke, and you should take steps to quit if you smoke. This can help slow the disease’s progression and ease your breathing.
  • Eat a well-balanced diet. Your doctor, nutritionist, or dietitian may recommend eating certain foods to help you maintain a healthy weight.
  • Follow an exercise plan developed with your doctor’s guidance. Exercise may help improve your lung function, strengthen your heart, and strengthen your muscles, which can help you maintain your energy levels.
  • Get adequate rest and avoid excess stress. Doing these things may help improve your breathing.

Younger people who don’t respond to other treatments might need a lung transplant.

What is the outlook for people with pulmonary fibrosis?

Outlook

The rate at which pulmonary fibrosis scars people’s lungs varies. The scarring isn’t reversible, but your doctor can recommend treatments to reduce the rate at which your condition progresses.

Many people with pulmonary fibrosis only live for about three to five years after they’ve been diagnosed, reports the American Lung Association. The condition can cause a number of complications, including respiratory failure. This happens when your lungs no longer work properly and they can’t get enough oxygen to your blood.

Pulmonary fibrosis also raises your risk of lung cancer.

Tips for prevention

Prevention

Some cases of pulmonary fibrosis may not be preventable. Other cases are linked to environmental and behavioral risk factors that can be controlled. Follow these tips to lower your risk of getting the disease:

  • Avoid smoking.
  • Avoid secondhand smoke.
  • Wear a face mask or other breathing device if you work in an environment with harmful chemicals.

If you’re having trouble breathing, make an appointment with your doctor. Early diagnosis and treatment can improve the long-term outlook for people with many lung diseases, including pulmonary fibrosis. While the disease can’t be cured, treatment may help slow its progression.

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