Interstitial Lung Disease Medications

Many medications are used to treat interstitial lung disease (ILD) and its various symptoms. The goals of medication treatment are to make breathing more productive, reduce inflammation, and suppress overactive immune systems.

 

Oral Corticosteroids

Prednisone or some other form of corticosteroid, is often the first medication used for many forms of ILD. For some people, corticosteroids alone may decrease lung inflammation and cause an improvement in symptoms. 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, psychosis, or hyper excitability, difficulty sleeping, tendency to develop diabetes, peptic ulcer, infections, cataracts and osteoporosis (weakening of the bones).

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

 

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. Myophenolate may produce side effects.

Some of the side effects include:

Abdominal distress (diarrhea, cramping and nausea), sleepiness, fatigue and muscle or joint pain.

 
Azathioprine (Imuran®)

Azathioprine is another medication that may be used to help reduce the amount of steroids required. It is used if the side effects from other medications are not tolerable. Azathioprine is not typically recommended for treatment of people with Idiopathic Pulmonary Fibrosis.

Some of the side effects include:

Fever, skin rash, abdominal distress (diarrhea, cramping and nausea) and blood disorders.

 
Cyclophosphamide (Cytoxan®)

Cyclophosphamide 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.

Some side effects include:

Abdominal distress (diarrhea, cramping and nausea), bladder inflammation, bone marrow suppression, infection, irregular menstruation and blood disorders.

 

Pirfenidone (Esbriet®)

Pirfenidone is an oral medication taken three times daily. The specific mechanism is unknown, but it seems to have anti-fibrotic and anti-inflammatory properties. Pirfenidone slows the progression of disease for some people with IPF.

Some side effects include:

Nausea, loss of appetite, stomach upset and photosensitivity (a tendency to easily develop sunburn, this may be severe).

 

Nintendanib (Ofev®)

Nintedanib is an oral medication taken twice daily. It is a triple kinase inhibitor that blocks several pathways that lead to the development of scars. Like pirfenidone, nintednib slows the progression of disease for some people with IPF.

Some side effects include:

Diarrhea and less often nausea and vomiting.

Because of the potential side effects of the above medications, your doctor will carefully monitor you while on therapy. This will include routine blood work. The decision to treat people with ILD 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 side effects.

 

 

This information has been approved by Gregory P. Cosgrove, MD, FCCP and Jeffrey James Swigris, DO, MS (December 2014).

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