Protein Helps Indicate Progression of Rare Lung Disease

Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with an expected survival time of about three to five years. Now, the link between a specific protein in the lungs and the progression of the illness may help doctors make better decisions about treatment methods for each individual patient.

The name of the protein is serum surfactant protein A, and doctors report that it is one of the best independent predictors of patients dying within the first year of IPF diagnosis. Head of research for the study, Dr. Brent W. Kinder of the University of Cincinnati Interstitial Lung Disease Center, reports that “this protein was more useful in predicting outcome than age, pulmonary function tests, or other conventional clinical markers of the disease.”

The new research suggests that a simple blood test to check for the presence of the protein can help determine the best course of treatment for IBF patients. For cases in which the protein suggests a short survival time, more informed decisions can be made about whether or not the undergo difficult treatment. Urgency of enrollment in clinical trials can also be gauged. In extreme cases, the new predictor may also help indicate the most optimal time to schedule a lung transplant.

IPF is a disease that results in scarring of the lung tissue. Overtime, healthy lung walls become replaced with fibrotic scar tissue. This fibrosis reduces the ability of the lungs to transfer oxygen into the bloodstream. This eventually leads to respiratory failure, heart failure, pulmonary embolism or similarly severe body malfunctions that result in death.

The new screening method is expected to assist in the roughly 50,000 new cases of IBF each year. The disease is most likely to affect individuals between the ages of 50 and 75.