Here are some medications commonly used in pulmonary fibrosis.  Not all individuals respond to medications in the same way or at all. 

Corticosteroids (Antifibrotic / Anti-Inflammatory Drugs)

Oral prednisone, or some other form of corticosteroid, is frequently the first medication used. For some people, corticosteroids alone may decrease lung inflammation and cause an improvement in symptoms. Other people may have to use steroids in combination with other therapies. It may take as long as three months to see results. Corticosteroids can have significant side effects.

Some of the side effects include:

  • Increased appetite
  • Weight gain
  • High blood pressure
  • Salt and fluid retention
  • Tendency to bruise easily
  • Depression
  • Hyperexcitability or psychosis
  • Tendency to develop diabetes
  • Peptic ulcers
  • Infections
  • Cataracts
  • Osteoporosis (a tendency to break bones)

Talk with your healthcare provider about preventing and watching for these side effects.

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Cytotoxic Agents or Immunosuppressive Drugs

Cyclophosphamide (Cytoxan®) may be used if steroid therapy has failed to be effective or if corticosteroid treatment is not possible. In some cases, a combination of a corticosteroid and cyclophosphamide is used with good results. This medication reduces inflammation by killing some inflammatory cells and suppressing their function. Response to therapy may be slow and require up to 6 months or longer. Cyclophosphamide can have significant side effects.

Some side effects of cyclophosphamide include:

  • Gastrointestinal irritation
  • Bladder inflammation
  • Bone marrow suppression
  • Infection
  • Irregular menstruation
  • Blood disorders

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Azathioprine (Imuran®)

Azathioprine is another drug often used in combination with corticosteroids for the treatment of pulmonary fibrosis. It is used if the side effects of other drugs are not tolerable. Though early studies show this drug may not be as effective, its side effects may be more manageable.

Some side effects of azathioprine may include:

  • Fever
  • Skin rash
  • Gastrointestinal irritation
  • Blood disorders

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Mycophenolate (CellCept®)

Mycophenolate can be used to help reduce the amount of steroids required. It works to prevent the immune system from attacking cells in the body that result in fibrosis. Mycophenolate may produce side effects.

The most common side effects include:

  • Abdominal distress
  • Sleepiness
  • Muscle or joint pain

Because of the potential side effects of the above medications, your doctor will carefully monitor you while on therapy. The decision to treat patients with pulmonary fibrosis involves a careful weighing of the potential risks and benefits of therapy. The potential benefits from the treatment usually outweigh the risk from the medication's side effects.

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This information has been approved by David Schwartz, MD (February 2010).