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 between 35.000 and 55.000 (13-20 cases/100.000 population). In the State of Alabama, the estimated prevalence is between 700 and 1000 cases. IPF is more common among elderly males.
How is it diagnosed?
Definitive diagnosis is made with a surgical lung biopsy showing “usual intersititial pneumonitis” without any significant history of exposure to certain chemicals/fibers or medications. Patients also cannot have any evidence of an auto-immune disease such as Rheumatoid Arthritis or Scleroderma. A “presumptive” diagnosis of IPF can be made based on history, physical exam findings, high resolution CT scan of the chest and, sometimes, smaller lung biopsies performed using a bronchoscope inserted through the patient’s nose or mouth. As stated before, however, only surgical lung biopsies can establish diagnosis without a doubt.
How is IPF treated?
The American Thoracic Society and the European Respiratory Society published the “International Consensus Statement on the Diagnosis and Treatment of IPF” in 2000 (see Useful links ). Essentially, the conventional approaches to the treatment include drugs with anti-inflammatory activity such as corticosteroids (prednisone) and immunosuppressive/cytotoxic drugs such as azathioprine (Imuran) and cyclophosphamide (Cytoxan). Unfortunately, such therapies were never shown to be capable to reduce the mortality of IPF. Lung Transplantation, for selected patients, remains the only modality of treatment that has been shown to prolong survival of patients with IPF. Multiple clinical trials (research protocols) using experimental drugs are on the way and it is now widely accepted that most patients with IPF should be considered for such trials whenever they qualify (see link Ongoing Clinical Trials to verify if you might qualify to be part of one of these studies). UAB has joined efforts with Tulane University and established the Gulf South IPF Research Center. Our center was selected with 10 others across the Nation to be part of the NIH IPF Clinical Research Network which will design and conduct research protocols seeking new treatment strategies for Idiopathic Pulmonary Fibrosis.
What is the prognosis?
Unfortunately, less than 10% of the patients will have some response to the conventional treatments described above and most will go on to have a progressive, downhill course. 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.