New hope for idiopathic pulmonary fibrosis patients?

When I was diagnosed with idiopathic pulmonary fibrosis in the fall of 1999 there was no hope for slowing or stopping the progression of the disease. The only option was a lung transplant.

The following news release describes a University of Pittsburgh study that suggests standard anti-inflammatory therapy may not be appropriate for many interstitial lung disease patients such as those with idiopathic pulmonary fibrosis (IPF) and a related study suggests that a protein known as osteopontin may be a target for therapy.

The investigators suggest that if their findings hold up in further, larger studies, doctors may be able to slow or stop the progression of this deadly disease. This is probably a long way from reality but at least there's a glimmer of hope for the future.

PITTSBURGH, Jan. 11, 2006 – In an article in the Jan. 15 issue of the American Journal of Respiratory Critical Care Medicine, University of Pittsburgh researchers report that a serious, life-threatening form of pulmonary fibrosis, called idiopathic pulmonary fibrosis, lacks all the hallmarks of inflammation and is probably unnecessarily treated with anti-inflammatory drugs. Moreover, in a related study, (see below) the investigators identified a protein found in excess amounts in the lung tissue of patients with idiopathic pulmonary fibrosis, which may be a more appropriate target for therapy.

Interstitial lung disease describes a diverse set of chronic lung conditions that often have strikingly similar symptoms but different clinical courses. However, all are characterized by differing degrees of progressive scarring of lung tissue between the air sacs, or the interstitium. With repeated damage, the interstitium becomes thickened and stiff, or fibrotic, making it increasingly difficult for the individual to breathe. Some forms of interstitial lung disease, particularly idiopathic pulmonary fibrosis, which has no known cause, have a very high death rate due to respiratory failure. Effective treatment, however, is complicated by the fact that a definitive diagnosis often requires a lung biopsy.

“Unfortunately, many patients do not receive lung biopsies. As a result, about one-third of patients who come to our clinic have previously been misdiagnosed, and many have been treated with the wrong medications,” explained James Dauber, M.D., medical director of the University of Pittsburgh’s Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, and professor of medicine, division of pulmonary, allergy and critical care medicine...Read the full news release


This release also refers to another study offering additional potential hope for IPF patients. It goes on to say:

"Although there currently is no effective treatment for idiopathic pulmonary fibrosis, results of another study suggest help may soon be on the way. Dr. Kaminski and his colleagues reported in the Sept. 6 online edition of PLoS Medicine that idiopathic pulmonary fibrosis lung tissue samples display an “over-abundance” of a protein known as osteopontin, which other studies have implicated in the growth and progression of tumors. In further examining the potential role of osteopontin in idiopathic pulmonary fibrosis, Dr. Kaminski’s group found that it directly increases the proliferation and movement of fibroblasts, cells centrally involved in lung fibrosis"... Full News Release