Pulmonary fibrosis symptoms, diagnosis and treatment - ABC 12 – WJRT – Flint, MI

By Louis Neipris, M.D., Staff Writer, myOptumHealth
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With each breath, millions of microscopic sacs in the lungs, called alveoli, fill with air. Tiny blood vessels between the alveoli pick up life-giving oxygen that travels in the blood to the brain and other organs of the body. Sometimes the delicate tissue that lines the alveoli becomes inflamed, thickens and develops into scar tissue. This process is called pulmonary fibrosis. Over time, this makes it hard for oxygen to enter the tiny blood vessels and get into the blood and out to the body.

What is pulmonary fibrosis?
Pulmonary fibrosis is a chronic condition. Its severity can vary greatly. For some, life expectancy is greatly reduced. For others, the disease progresses more slowly or stays the same for many years.

Most cases of pulmonary fibrosis have no clear cause and are called "idiopathic" (idiopathic pulmonary fibrosis). It is likely that some ongoing injury to lung tissue leads to inflammation and scarring.

There are some clear risks factors for developing pulmonary fibrosis, including:

• Cigarette smoking.
• Gastroesophageal reflux disease (GERD). In GERD, the stomach acid backs up into the food pipe (esophagus) and throat.
• Environmental exposure to toxins and pollutants, such as inorganic dust (asbestos, and silica) as well as organic dust from airborne plant and animal material.
• Certain viral infections, such as Epstein-Barr virus (EBV), which cause mononucleosis, influenza A virus and hepatitis C.
• Family history. There is a genetic link in pulmonary fibrosis.
• Chemotherapy and radiation treatment to the chest.

What are the symptoms?
The two most common symptoms are:

• Shortness of breath, which at first may be only during exercise. Over time, shortness of breath occurs at rest.
• Dry hacking cough, which may get worse.

Other symptoms are:

• Weight loss
• Fatigue
• Aching muscles and joints
• Rounding and widening of the tips of the fingers and toes (clubbing)

How is it diagnosed?
Your doctor will ask about your symptoms, when they began and how they interfere with your life. You'll have a complete physical exam, and the doctor will listen closely to your lungs with a stethoscope as you take deep breaths through your mouth. Your doctor may order additional diagnostic tests or procedures that may include:

• Chest x-ray. Images of the lung structures may reveal patterns of scarring that occur with pulmonary fibrosis.
• CT scan (computed tomography). This imaging technique produces detailed images of lung tissue. It can sometimes diagnose the disease at an early stage and help predict response to treatment.
• Pulmonary function tests. In this test, you breathe through a mouthpiece into a machine that measures how much air your lungs can hold and how quickly you can move air in and out of your lungs.
• Pulse oximetry. A small infrared sensor, clipped to the tip of your finger, measures the percentage of oxygen (percent saturation) of your blood.
• Lung biopsy. In a biopsy, a tissue sample is examined under the microscope. Examination of lung tissue provides a definitive way to diagnose pulmonary fibrosis. A biopsy can help doctors tell the difference between pulmonary fibrosis and other diseases, such as emphysema, that can cause similar symptoms.

How is it treated?
There is no treatment that can reverse the scarring that has already occurred. But medicines can help slow the process, relieve symptoms and improve your quality of life. Treatments include:

Medications. Treatment usually includes the steroid prednisone to reduce inflammation and an immunosuppressive drug, such as azathioprine or cyclophosphamide. Taking a combination of prednisone and an immunosuppressive drug requires careful monitoring for serious side effects, which may include:

• Infections
• Diabetes
• Glaucoma
• Reduction in number of blood cells
• Skin cancer
• Lymphoma

Research is ongoing to find more effective drugs and to determine why some people respond to medication and others don't.

Oxygen therapy. Supplemental oxygen may be prescribed if the amount of oxygen in your blood is low. Oxygen therapy may help reduce shortness of breath and make it easier for you to stay active. There are portable devices that allow you to carry oxygen around with you throughout the day.

Lung transplant. If medications are not helping, you may be referred for a lung transplant. Lung transplants may be advised for those who are under age 65 and have no other medical problems. A transplant can lengthen life, but requires taking medications for life to prevent infection and organ rejection. It may take months or even years to receive a donor lung because the supply of donor lungs is limited. So getting on the donor list as soon as you are eligible is very important.

What else do I need to know about pulmonary fibrosis?

• Get your flu and pneumonia vaccines. These vaccines help to prevent infections, which can be very serious if you already have lung disease.
• Take care of bone health. If you are taking prednisone, or another corticosteroid, talk to your doctor about taking calcium supplements and vitamin D. Your doctor may also suggest another medicine to build bone mass if you have thinning of the bones (osteopenia or osteoporosis).
• Consider pulmonary rehabilitation (pulmonary rehab). In rehab, you learn about your condition, how to exercise and live with the disease while finding support.
• Consider investigational treatments. Ask your doctor about taking part in a clinical trial where a new drug is being used to treat pulmonary fibrosis. You can also find information on clinical trials on the National Institutes of Health clinical trial database on the Web.

SOURCES:

• American Lung Association. Pulmonary fibrosis. Accessed: 02/08/2010

• National Jewish Health. Interstitial lung disease (ILD): overview. Accessed: 02/08/2010

• American Thoracic Society and the European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment international consensus statement. American Journal of Respiratory and Critical Care Medicine. 2000;161(2):646-664.

• National Heart Lung and Blood Institute. Idiopathic pulmonary fibrosis, scarred lung tissue. Accessed: 02/08/2010

• Coalition for Pulmonary Fibrosis. Frequently asked questions about pulmonary fibrosis. Accessed: 02/08/2010

• Noth I, Martinez FJ. Recent advances in idiopathic pulmonary fibrosis. Chest. 2007;132(2):637-650.