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What is the treatment for pulmonary fibrosis?

The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing.

Two drugs, nintedanib (Ofev) and pirfenidone (Esbriet, pirfenex, Pirespa) have offered some new hope. The results from two large studies show some slowing of progression of fibrosis, as well as some minor changes to tests of lung function. Unfortunately, the side effects from both of these drugs have resulted in some patients being unable to take this medication.

Since some types of lung fibrosis can respond to corticosteroids (such as prednisone) or other medications that suppress the body's immune system; these types of drugs sometimes are prescribed in an attempt to decrease the processes that lead to fibrosis. These drugs do not help idiopathic pulmonary fibrosis. However, other causes of lung fibrosis may be responsive to immune suppression.

The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Conditions that improve with immune suppressive treatment probably are not idiopathic pulmonary fibrosis.

The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need and duration of treatment, and will monitor the response to therapy along with any side effects.

Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids especially if a different cause for interstitial lung disease is suspected. These include:

  • cyclophosphamide (Cytoxan)
  • azathioprine (Imuran, Azasan)
  • methotrexate (Rheumatrex, Trexall)
  • penicillamine (Cuprimine, Depen)
  • cyclosporine

The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon and mycophenolate mofetil (Cellcept) have not met with much success in the treatment of idiopathic pulmonary fibrosis.

Pulmonary fibrosis causes decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen therapy to prevent pulmonary hypertension.

A consensus statement was issued revising the 2011 clinical practice guidelines for the treatment of idiopathic pulmonary fibrosis. This statement was issued by one the largest respiratory physician groups in the world, including members from the United States, Europe, Japan, and Latin America. These recommendations are partially based on a recent study involving the use of prednisone, azathioprine, and N-acetylcysteine, so called triple therapy. In this study, drug recipients had a 10 fold increase in the death rate than the placebo group. These new guidelines therefore strongly discourage the use of triple therapy in idiopathic pulmonary fibrosis. These guidelines also suggest that anticoagulation and anti GERD (gastroesophageal reflux disease) not be administered for idiopathic pulmonary fibrosis alone. These medications should not be used without other medical indications in patients with idiopathic pulmonary fibrosis. Lastly, patients with idiopathic pulmonary fibrosis often have pulmonary hypertension as their disease progresses. There is currently no indication to use pulmonary hypertensive medication in these patients except oxygen in those with low oxygen levels.

Return to Pulmonary Fibrosis

See what others are saying

Comment from: Linda, 65-74 Female (Patient) Published: September 29

I have polymyositis, been also diagnosed with pulmonary fibrosis, and I'm on oxygen at this time. I'm on prednisone, a very high dose! I'm taking a shot three times a week plus I'm to go in once a month to the hospital to give my immune system a boost of new cells. I have to say if I received the help when I was in my thirties things might have been different. I've been fighting depression and don't know how to bring myself out of it. I am also in a wheel chair most of the time, so finding things to do is limited. I try every day to have a smile on my face, but it's hard! I getting counseling soon!

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Comment from: Renee, 55-64 Female (Patient) Published: July 09

I was diagnosed with polymyositis in 2002 and idiopathic pulmonary fibrosis (IPF) by a lung biopsy in 2007. I have been on prednisone at various dosages from the beginning along with various immune suppressant medications. I began taking tacrolimus in 2008 and have held off any progression of more scarring. I have shortness of breath and coughing. I am relatively active but I have learned to pace myself. I have the best medical team that all work together.

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Comment from: dondee60p, 65-74 Female (Patient) Published: June 20

I was diagnosed with pulmonary fibrosis (PF) in 2002. I have had pneumonia many times, caused by severe GERD. The doctor did a Nissen fundoplication which kept me from aspirating into my lungs. I take prednisone 10 mg daily, more if sick. I also take NAC (N-acetyl cysteine), 600 mg 3 times/day. I have been stable for several years and only need to see my pulmonologist once a year, or if necessary. My pulmonologist told me he doubted I would die from PF as another disease would take me first. That made me very happy! I do continue to hope for others with IPF or PF that there will be a cure soon.

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