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)
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.