Medical Edge -- Treating pulmonary fibrosis can be a challenge

Tribune Media Services

DEAR MAYO CLINIC: I’ve had numerous bouts of pneumonia over the years. Now I’ve been told that I have pulmonary fibrosis. Can you tell me what to expect and is there anything I can do?

Pulmonary fibrosis is a scarring process in the lungs that tends to develop slowly over a period of years. It’s often initially diagnosed incorrectly as an infection or pneumonia, which may have been the case in your situation. Getting an accurate diagnosis and finding out what’s causing the condition are critical to effectively managing pulmonary fibrosis and conditions related to it.

When you breathe in, tiny air sacs in your lungs (alveoli) fill with air. These sacs are richly supplied with blood. The alveoli allow oxygen from the air to get into your blood, and carbon dioxide to get out of your blood and into your lungs, so you can breathe it out.

In pulmonary fibrosis, the alveoli slowly fill with scar tissue. Some stop working completely. Within the alveoli that continue to function, oxygen and carbon dioxide have to travel through scar tissue to get into the bloodstream or into the air. The lungs become stiff, and breathing becomes rapid and shallow. Because oxygen and carbon dioxide don’t exchange well through the scarred lung tissue, blood oxygen levels eventually drop.


Diagnosing pulmonary fibrosis can be difficult, because its symptoms — shortness of breath that gets progressively worse, a persistent, dry cough and, in some cases, joint achiness — are nonspecific and can be caused by many other conditions. A chest X-ray of someone who has pulmonary fibrosis usually shows shadows in the lungs, which may be misidentified as pneumonia.

A pulmonologist — a physician who specializes in diseases of the lung — who is experienced with pulmonary fibrosis can accurately diagnose the disorder through a series of X-rays, CT scans, and, on occasion, a lung biopsy. After diagnosis, the next step is identifying the cause. This can also prove difficult because fibrosis in the lungs has more than 250 causes. It’s imperative to seek care from a specialist who is familiar with pulmonary fibrosis and can determine if your condition is caused by a factor that is reversible.

For example, in some people, certain drugs can cause pulmonary fibrosis. These include some chemotherapy drugs, certain heart medications and, occasionally, antibiotics. If your doctor identifies a drug as the source of the problem, an alternative can be prescribed, and the fibrosis may stabilize or improve. In other cases, exposure to a dust, such as asbestos, or to certain microbes or allergens, can cause lung scarring. If the substance causing the reaction is removed, the progress of fibrosis usually can be stopped.

Smoking has not been proven to directly cause pulmonary fibrosis, but smokers have a higher rate of the disease than nonsmokers. Also, in a few cases, genetics may play a role.

Although some causes of pulmonary fibrosis can be identified, many cannot. In these cases, where the cause is unknown (idiopathic), the disease progression usually continues. How fast pulmonary fibrosis progresses varies among individuals, but ultimately it can be fatal. Treatments that may help slow the advance of the disease or reduce symptoms include medications that can help reduce the development of scar tissue (anti-fibrotic agents) and, in some cases, medications that suppress the immune system. A lung transplant may also be a treatment option, particularly for younger people who are otherwise in good health.

Even though idiopathic pulmonary fibrosis has no known cure in 2008, more clinical research studies and new medications have been advanced for the treatment of pulmonary fibrosis in the past several years than I have seen in the previous 20 years.

If the cause of your condition can’t be determined and treated, I recommend you contact a pulmonologist at an academic medical center to find out if you’re eligible to participate in a research study about pulmonary fibrosis. A clinical trial may offer you the best, most current treatment available to combat this serious condition. — Andrew Limper, M.D., Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester.

E-mail a question to or write: Medical Edge from Mayo Clinic, c/o TMS, 2225 Kenmore Ave., Suite 114, Buffalo, N.Y., 14207.

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