Fibrosis File

A recent lecture at the American College of Chest Physi- cians conference discussed a theory that Idiopathic Pulmonary Fibrosis (IPF) is caused by a repeated stimulus to the lung which results in injury. The injured lung does not heal properly and fibrosis or scarring occurs. The part of the lung that is fibrotic can not expand to take a deep breath or participate in the exchange of carbon dioxide and oxygen. People who have a “vulnerable lung” and/or predisposing genetic factors are often the victims of IPF. Smokers and those with rheumatoid arthritis are more likely to have the disease.

Current clinical trials will compare people with early stage IPF who take azathioprine (an immunosuppressant), prednisone (a corticosteroid) and N-acetyl cysteine (NAC) to those who take NAC only and to those who are given a placebo. NAC is an amino acid that serves as a precursor for the synthesis of glutathione, a detoxifying agent in the body. If treatment groups are no better than giving a placebo, we can stop giving the medications which are known to have many side effects. Another study involving people with moderate to severe IPF will compare people who take sildenafil (Viagra) or a placebo. Physicians are researching giving Warfarin

(Coumadin) in addition to corticosteroids to see if it will be beneficial in IPF. Using DNA chips, University of Pitts- burgh scientists plan to examine the activity of people’s genes to find out more about the links in two of the most common cigarette-associated lung diseases: COPD and IPF.

We now only have supportive therapy, not curative. Scientists are working to find out what causes an acute exacerbation (worsening of the disease), why is smoking such a risk factor and why does IPF develop after the person stops smoking? We do know collapse of airways with abnormal re-expansion results in fibrosis. We know fibrosis is related to aging and those with IPF have short- ened telomeres (DNA at the end of chromosomes which protect the cells from destruction). Your physicians are anxiously awaiting the latest news to help you!

A University of Pittsburgh team is the first to receive approximately $3 million in federal money to use “biochip” technology to study different smoking related chronic lung diseases. Using DNA chips, Pitt scientists plan to examine the activity of people’s genes to find out more about genetic links in COPD and IPF.

“This study will help us understand why one person responds to cigarette smoke by developing emphysema while another develops fibrosis, and then to rapidly translate this knowledge from bench to bedside,” said Dr. Naftali Kaminski, director of Pitt’s Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases.