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Medical Author:

George Schiffman, MD, FCCP

George Schiffman, MD, FCCP

Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

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Medical Editor:

Melissa Conrad Stöppler, MD

Melissa Conrad Stöppler, MD

Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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In this Article

• What is pulmonary fibrosis?
• What are causes and symptoms of pulmonary fibrosis?
• How is pulmonary fibrosis diagnosed?
• How is pulmonary fibrosis treated?
• What are the complications of pulmonary fibrosis?
• Can pulmonary fibrosis be prevented?
• What is the prognosis for pulmonary fibrosis?
• Where can I get information to improve the quality of life for those with pulmonary fibrosis?
• Patient Comments: Pulmonary Fibrosis - Experience
• Patient Comments: Pulmonary Fibrosis - Symptoms
• Patient Comments: Pulmonary Fibrosis - Treatments
• Find a local Pulmonologist in your town
• Pulmonary Fibrosis Index

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.

Since 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. These drugs do not help idiopathic pulmonary fibrosis but in some cases, other causes of lung fibrosis responsive to immune suppression may mimic the appearance of idiopathic pulmonary fibrosis.

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 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 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

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

The anti-inflammatory medication colchicine has also been used with limited success. Other trials using newer drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone 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 to prevent pulmonary hypertension. In some cases, new agents used to lower the blood pressure in the pulmonary artery have had limited benefit.

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.