Pulmonary Fibrosis

Pulmonary fibrosis is the end stage of interstitial lung disease, a large group of disorders that cause progressive lung scarring. The current thinking is that pulmonary fibrosis begins with repeated injury to the lining of the alveoli, the small air sacs in your lungs. The damage eventually leads to scarring (fibrosis), which stiffens your lungs and makes breathing difficult.

No cure exists for pulmonary fibrosis, and current treatments often fail to slow the progress of the disease or relieve symptoms. A number of new therapies are in clinical trials, however, and researchers hope that better treatments will become available. In the meantime, a lung transplant may be an option for some people with pulmonary fibrosis.

Signs and Symptoms

The most common pulmonary fibrosis symptoms are shortness of breath (dyspnea), especially during or after physical activity, and a dry cough. Unfortunately, these often don’t appear until the disease is advanced, and irreversible lung damage has already occurred. Even then, you may downplay your symptoms, attributing them to aging, being out of shape or the lingering effects of a cold.

But breathing problems usually become progressively worse, and eventually you’re likely to get out of breath during routine activities — getting dressed, talking on the phone, even eating. At this point, symptoms are impossible to ignore.

You may also notice other signs and symptoms, including:

  • Fatigue
  • Unexplained weight loss
  • Aching muscles and joints

Pulmonary fibrosis can vary considerably from person to person. Symptoms range from moderate to severe. Some people become ill very quickly, whereas others grow worse over a period of months or years.

Source: Mayo Clinic

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6 Comments so far

  1. James David | 03 January 2010, 17:53

    Is there a network blog of patients with pulmonary fibrosis who have taken pirfenidone (pirespa) for this condition. I would like to know their personal experience and which doctors are overseeing their treatment.

  2. Gay Mitchell | 08 February 2010, 23:17

    I’d like to share with other IPF patients. Have tons of questions. Need honest answers.

  3. Marian Stillman | 30 May 2010, 00:15

    What is making a cracking noise in my lungs. I was told that I may have a form of pulinary fibrosis. I had by-pass surgery mostly in the pulminary arties . I have fatigue, and sleep more than usual and have ra and diabetses

  4. lynda whiteman | 30 August 2010, 12:17

    my husband was diagnosed with pulmonary fibrosis in November 2009. He has been doing good. the past two months his coughing is getting bad, bad enough to keep him sleeping at night. I recently notice streaks of blood in the mucus. what does that mean?

  5. lynda whiteman | 30 August 2010, 12:17

    my husband was diagnosed with pulmonary fibrosis in November 2009. He has been doing good. the past two months his coughing is getting bad, bad enough to keep him from sleeping at night. I recently notice streaks of blood in the mucus. what does that mean?

  6. Anne Cooper | 30 August 2010, 12:46

    If your husband was diagnosed with pulmonary fibrosis in November 2009, and he is currently having persistent cough, and most especially that he has blood in his mucus, this indicates that his condition is worsening and may indicate that irreversible lung damage may have already been done. The best thing to do right away is to consult your healthcare provider, and inquire about ordering a chest x-ray, pulmonary (lung) function tests, and perhaps, a bronchoscopy. I hope this helps.