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Lung Disease & Respiratory Health Center

Pulmonary Fibrosis

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How Is Pulmonary Fibrosis Treated?

The treatment options for idiopathic pulmonary fibrosis are limited. Lung transplantation is the only therapeutic option available, but this is usually reserved for advanced cases. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. 

Two drugs, Esbriet (pirfenidone) and Ofev (nintedanib), are FDA-approved to treat idiopathic pulmonary fibrosis. They act on multiple pathways that may be involved in the scarring of lung tissue. Studies show both medications slow decline in those with the disease when they're given breathing tests. 

Other research trials using different drugs to decrease lung scarring are ongoing. Because some types of lung fibrosis can respond to corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis. When these fail, lung transplantation may be considered for some patients.

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. Those whose conditions improve with immune suppressive treatment probably do not have idiopathic pulmonary fibrosis.

The toxicity and side effects of treatment 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 for treatment, the 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. These include gamma-interferon, cyclophosphadmide, azathioprine, methotrexate, penicillamine, and cyclosporine. The anti-inflammatory drug colchicine has also been used with limited success. Ongoing trials are under way using newer drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone, but results so far have been inconclusive.

Pulmonary fibrosis can cause 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 to prevent pulmonary hypertension.

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