Idiopathic Pulmonary Fibrosis Treatment

Corticosteroids
The mainstay of treatment for idiopathic pulmonary fibrosis is medication that helps to reduce inflammation. Inflammation is thought to play a role in the injury and fibrosis of the lung tissue. Therefore, medicines that stop the inflammation are thought to have an effect on the resulting damage.
 
The anti-inflammatory medicine that most healthcare providers prescribe for idiopathic pulmonary fibrosis is high-dose prednisone. Prednisone is part of a group of medications known as glucocorticoids, corticosteroids, or simply "steroids" for short. People who have IPF usually take prednisone by mouth every day.
 
Because prednisone can cause serious side effects (see Prednisone Side Effects) and does not work in many cases, a healthcare provider may only prescribe it for three to six months. Then, if improvement is seen, he or she may reduce the dose over time and keep the person on it for a longer period of time (this is known as a maintenance dose).
 
Immune Suppressants
Many healthcare providers also prescribe a second medicine with prednisone, such as azathioprine (Imuran®) or cyclophosphamide (Cytoxan®). These medicines can be prescribed alone if a person does not respond well to prednisone or develops serious side effects.
 
Azathioprine is a medicine that affects the immune system. Most people take it by mouth every day. Because it can cause serious side effects (see Side Effects of Azathioprine), a healthcare provider may prescribe it with prednisone for only three to six months. Then, if the person doesn't have serious side effects and the drug combination seems to help, the healthcare provider may prescribe it for a longer period of time.
 
Cyclophosphamide is another immune system suppressant that healthcare providers use to treat IPF. They usually prescribe it in addition to low doses of prednisone for people who are getting worse while only taking prednisone. Many people who can't take prednisone can take cyclophosphamide by itself.
 
Most people take cyclophosphamide by mouth every day. Some people with IPF receive it for three to five days through a needle that is inserted into a vein in the arm. After that, they take it by mouth every day. You usually start on a low dose, which is then increased over time. It may take three to six months before you see any benefits from cyclophosphamide. It can also cause serious side effects (see Cyclophosphamide Side Effects).

IPF Information

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