Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) occurs when the lung tissue becomes scarred. This scarring causes the lung tissue to become less elastic and less efficient at gas exchange. As it advances, you will have to breath faster to compensate for reduced lung volumes. This becomes a dangerous cycle as you will need to work harder to breath while at the same time not be able to meet the oxygen demands of your tissues due to damage of the alveoli (tiny air sacs) in the lungs. IPF is the most common form of interstitial lung disease, a group of diseases that affect the tissue between the airways and bloodstream through both lungs.

SYMPTOMS

The most common symptoms of IPF are shortness of breath and a dry, persistent cough. Other symptoms associated with the condition include:

Fast, shallow breathing
Gradual weight loss
Tiredness
Aching joints and muscles
Clubbing (widening and rounding of the tips of the fingers or toes)

CAUSES

Pulmonary fibrosis is linked to many conditions, but in most cases, the cause is never found. When no cause is known, the condition is called idiopathic pulmonary fibrosis. Researchers are looking into what might cause the disease. Viruses and tobacco smoke are two possible causes. Certain types of the disease seem to run in families. The condition can affect both men and women and is most often diagnosed in patients between the ages of 50 and 70 years old.

DIAGNOSIS

Diagnosis of pulmonary fibrosis begins with a history and physical exam. Additional tests may include:

Chest X-ray: This imaging test takes a picture of the heart and lungs. Pulmonary Fibrosis may show as an increased tissue density resulting from areas of inflammation or fibrotic changes.
CT scan: This imaging test uses X-rays and computer technology to make horizontal images (called slices) of the body. A CT scan may be ordered if the chest X-ray showed anything that warranted further investigation. CT scans provide more detailed information.
Pulmonary function tests: This test can be performed in the office. This test measures several different aspects of the lungs. Pulmonary Fibrosis will often show reduced lung volumes, and if more advanced will additionally show decreased gas exchange.
Arterial blood gas: This test can assess how well the lungs get oxygen into the blood stream and carbon dioxide out of the blood stream. As Pulmonary Fibrosis progresses an impairment of gas exchange may be noted in this test.
Bronchoscopy: A long, thin, flexible tube (bronchoscope) with a light at the end is put down the throat and into the lungs. This allows the physician to directly view different parts of the lungs. If the physician sees something that warrants further investigation, they can obtain a tissue sample called a biopsy. This sample can then be sent to pathology where it will be tested.
Lung biopsy: A small piece of tissue, cells, or fluid from the lungs is taken out and checked under a microscope.
Sarcoidosis is often diagnosed when other lung disorders are ruled out.
Bronchoalveolar lavage: During a bronchoscopy the physician may use sterile saline to cleanse areas of the lung. This saline is then sucked back out and can be sent for testing. The saline carries out cells from the lower respiratory tract. These cells can be checked under a microscope to help find inflammation and infection. The test can help rule out certain causes.

TREATMENT

There is no cure for Pulmonary Fibrosis. Treatments are geared toward minimizing symptoms. Treatments include:

Steroids: Help to reduce inflammation by suppressing the immune system.
Oxygen therapy: If your oxygen saturation (SPO2) is low, supplemental oxygen may be prescribed to help get your SPO2 into the normal range.
Flu and pneumonia vaccine: Because Pulmonary Fibrosis increases your risk for severe illness, these yearly vaccines are recommended to take preventatively.
Reflux treatment: Reflux can cause Pulmonary Fibrosis symptoms to worsen. Treatment with proton pump inhibitors (PPI's) or histamine-2 agonists (H-2 blockers).
Pulmonary Rehab: If you have a Pulmonary Fibrosis and symptoms are worsening, you may qualify for pulmonary rehab. Pulmonary rehab takes place in a fitness center/gym setting and is overseen by medical professionals. They will help you determine the proper place to start, in terms of activity and intensity level, and help you make a plan to improve your cardiopulmonary performance. Not only does this help to optimize your lung function while attending, but gives you the tools to maintain that function after it has ended.
Lung transplant: This is a consideration for people with end-stage Pulmonary Fibrosis.