What is Idiopathic Pulmonary Fibrosis (IPF)?
IPF is a chronic, progressive, lung disease characterized by disabling shortness of breath, declining exercise capacity and extensive formation of scar tissue (fibrosis) in the lung.
Is there a specific cause?
No, IPF is a clinical diagnosis of exclusion of other conditions that may cause scar tissue formation in the lungs, such as autoimmune disorders (rheumatoid arthritis, for example) or occupational exposure (asbestos, silica) among others. There a number of reports attempting to link the development of IPF to viruses and some bacteria as well as supporting the role of genetic factors, however the data are still not conclusive.
How common is IPF?
The estimated prevalence of IPF in the United States is about 40/100.000 population. IPF is more common among elderly Caucasian males.
How is it diagnosed?
Definitive diagnosis is made with a surgical lung biopsy showing "usual interstitial pneumonia" without any significant history of exposure to certain chemicals/fibers or medications. Patients also cannot have any evidence of an autoimmune disease such as Rheumatoid Arthritis or Scleroderma. Often, the findings of a high resolution CT scan of the chest may be typical enough so that a lung biopsy is not necessary for the diagnosis of IPF, provided there are no other identified possible causes such as occupational exposures or auto-immune disease.
How is IPF treated?
There are no approved treatments for IPF. Lung Transplantation, for a selected few, remains the only modality of treatment that has been shown to prolong survival of patients with IPF. Pulmonary Rehabilitation has been shown to improve quality of life and endurance of patients with IPF. Because no effective treatments are available, most experts recommended that patients with IPF should be enrolled in clinical trials. The UAB ILD Program is part of the NIH-funded IPF Clinical Research Network (IPFnet). The IPFnet is comprised of 22 centers of excellence in the diagnosis and treatment of IPF. Its mission is to find effective therapies for patients with both the early and advanced stages of IPF.
What is the prognosis?
About 50% of the patients will die within 3 to 5 years from the diagnosis.
Will I become "dependent" on supplemental oxygen?
All human beings need oxygen to survive. The air has approximately 21% of oxygen in it. Some patients, especially those with more advanced disease, need to inhale higher oxygen concentrations to achieve adequate blood levels. If your doctor detected low oxygen levels at rest or exercise, you should use supplemental oxygen as directed. It is beneficial and may spare your heart, brain and other vital organs from damage caused by low blood levels of oxygen.
Will I have a lung transplant evaluation?
If we think you may benefit from the procedure, we will make you an appointment with our Lung Transplantation Program. Many patients, because of other diseases such as kidney or heart failure as well as advanced age or poor physical conditioning are not likely to benefit from lung transplantation.
How can I get involved in experimental protocols?
After our evaluation is finished, including review of your lung biopsy, we will contact you and discuss any ongoing clinical trials for which you may qualify.