Idiopathic pulmonary fibrosis

Lung transplantation for idiopathic pulmonary fibrosis (IPF) has been shown to have a survival benefit over medical therapy. IPF is now the most common indication for lung transplantation in the United States. Any person diagnosed with IPF should be referred for lung transplantation evaluation. A 2009 retrospective review of UNOS data found that the reported 5-year survival rates after lung transplantation in IPF were estimated at 50-56%. This is greater than the 20-30% general 5-year survival rate for affected people.

It is somewhat controversial whether people with IPF should have a single or bilateral lung transplant. Some have reported no difference in overall survival between the two types of transplants. Others have found a trend toward better early survival after a single transplant, and better long term survival after a bilateral transplant. It has now been seen in several studies that bilateral transplants may have better long-term outcomes, but worse short-term outcomes; single transplants have been associated with a slightly higher later risk of death.

Although the question of whether to perform a single or bilateral transplant remains unanswered, there is an increasing tendency in the transplant community to perform bilateral rather than single lung transplants. The data overall seem to suggest that although bilateral lung transplant may increase the risk for early death, it promotes long-term survival. It is possible that the early mortality caused by bilateral lung transplants has been improved by better surgical techniques and critical care.

There may be a number of research studies investigating new therapies for the treatment of idiopathic pulmonary fibrosis. Click here to learn more about how to get involved in research.

Idiopathic pulmonary fibrosis (IPF) is a condition in which tissues in the lungs become thick and stiff, or scarred, over time. The lungs then lose their ability to move oxygen to the brain and other parts of the body. Common symptoms include shortness of breath and a dry, hacking cough. In some cases fibrosis happens quickly, while in others, the process is much slower. Sometimes the disease stays the same for years. The condition is 'idiopathic' because the cause is unknown. When multiple family members are affected, it is called familial IPF. Many people with this condition live for about 3-5 years after the diagnosis. The most common cause of death is respiratory failure.

The long-term outlook (prognosis) for people with idiopathic pulmonary fibrosis (IPF) is poor, with only 20-30% of affected people surviving at least 5 years after diagnosis. Several factors have been associated with a shortened survival time, including:

• older age at diagnosis
• extensive cigarette smoking
• lower body mass index (BMI)
• more severe physiologic impairment
• greater extent of disease on imaging studies
• the development of other complications or conditions (eg, pulmonary hypertension, emphysema, and bronchogenic cancer)

The natural history of IPF is not completely understood. While it usually follows a course of progressive deterioration, some people remain stable for extended periods and individual outcomes can vary significantly. Still, long- term survival of IPF is not expected.

It now appears that three potential disease courses exist, but there is not currently a definitive way to predict the course in an affected person. They are:
a) slowly progressive disease (the most common);
b) disease marked by episodes of acute exacerbations; and
c) rapidly progressive disease.

In the past, the goals of treating idiopathic pulmonary fibrosis (IPF) have been to prevent more lung scarring, relieve symptoms, maintain the ability to be active, and improve the quality of life. More recently, pirfenidone (an anti-fibrotic drug) has been approved to treat people with mild-to-moderate IPF in the European Union, Canada, and Asia. The U.S. Food and Drug Administration (FDA) has granted Breakthrough Therapy Designation for pirfenidone and nintedanib, due to trials suggesting they slow the progression of IPF. Several other drugs are being studied as potential treatments including cotrimoxazole, thalidomide, sildenafil, andimatinib mesylate. However, more research is needed to determine their safety and effectiveness.

Most affected people need oxygen therapy at some point to increase oxygen levels in the bloodstream. Oxygen therapy can reduce breathlessness and allow people to be more active. Some people benefit from pulmonary rehabilitation, used for people with chronic lung diseases.

People with IPF may eventually need a lung transplant. This is more likely in younger patients (under 65) with severe disease who have not responded to other treatments, and who don't have other serious medical problems. Some consider lung transplants for people over 65 who don't have other serious medical problems.

For many years, corticosteroids (such as prednisolone) along with immunosuppressive drugs (such as azathioprine) were used to treat IPF. Sometimes an additional drug called N-acetylcysteine has also been used. These drugs were recommended based on the theory that generalized inflammation was a major part of IPF. However, the drugs were often ineffective and there has not been evidence that they improve long-term survival.

Gastroesophageal reflux may be treated with standard medications. Some studies have shown longer survival times and lower fibrosis scores in people receiving treatment for gastroesophageal reflux.