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Pulmonary Fibrosis

How Is Pulmonary Fibrosis Treated? continued...

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.

There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated.

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WebMD Medical Reference

Reviewed by Kimball Johnson, MD on July 04, 2012

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