Pulmonary fibrosis

DEFINITION

Pulmonary fibrosis occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.

The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can’t pinpoint what’s causing the problem. When a cause can’t be found, the condition is termed idiopathic pulmonary fibrosis.

The lung damage caused by pulmonary fibrosis can’t be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.

CAUSES

Pulmonary fibrosis scars and thickens the tissue around and between the air sacs (alveoli) in your lungs. This makes it more difficult for oxygen to pass into your bloodstream. The damage can be caused by many different things — including airborne toxins in the workplace, certain lung diseases, radiation directed to cancers in the lung or breast, and even some types of medical treatments.

Occupational and environmental factors

Long-term exposure to a number of toxins and pollutants can damage your lungs. These may include:

  • Silica dust
  • Asbestos fibers
  • Grain dust
  • Bird and animal droppings



Radiation treatments

Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage depends on:

  • How much of the lung was exposed to radiation
  • The total amount of radiation administered
  • Whether chemotherapy also was used
  • The presence of underlying lung disease



Medications

Many drugs can damage your lungs, especially:

  • Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Trexall) and cyclophosphamide (Cytoxan), can also damage lung tissue.
  • Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Cordarone, Nexterone, Pacerone) or propranolol (Inderol , Innopran), may harm lung tissue.
  • Some antibiotics. Nitrofurantoin (Macrobid, Macrodantin, others) and sulfasalazine (Azulfidine) can cause lung damage.



Medical conditions

Lung damage can also result from:

  • Tuberculosis
  • Pneumonia
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Sarcoidosis
  • Scleroderma



The list of substances and conditions that can lead to pulmonary fibrosis is long. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.

Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. And because one type of idiopathic pulmonary fibrosis runs in families, heredity also is thought to play a role.

SYMPTOMS

Signs and symptoms of pulmonary fibrosis include:

  • Shortness of breath (dyspnea)
  • A dry cough
  • Fatigue
  • Unexplained weight loss
  • Aching muscles and joints

The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.