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Fall 2006

Diagnosis Crucial Step in Treating Pulmonary Fibrosis

Seeking care at a medical center that offers both lung transplantation and clinical trials of new pharmacologic therapies is strongly recommended for patients with most forms of pulmonary fibrosis. Transplantation is the only known-to-be-effective option currently available to people with the most common form of the disease, which has a median survival of three to four years from the time of diagnosis.

Pulmonary fibrosis is a condition characterized by scarring of the lung. As the lung's air sacs are gradually replaced by fibrotic, or scarred, tissue, the ability to transfer oxygen to the bloodstream is affected, causing a shortness of breath that gets worse over time. The prognosis varies. Certain forms can progress slowly, but with idiopathic pulmonary fibrosis, the major form of the disease -for which the cause is unknown-the prognosis is poor and treatment options are limited.

"Typically, drug therapy comes with significant side effects and doesn't change the course of the disease or extend survival," says Joseph Lynch, M.D., UCLA pulmonologist. Although some new drugs are being tested at UCLA and other major centers through clinical trials, Dr. Lynch notes that the best current option for patients with idiopathic pulmonary fibrosis or other rapidly progressive forms of the disease is transplantation. "Lung transplantation is life-saving," Dr. Lynch says. "Patients can have complete restoration of their lung function following transplant." But because the disease is relatively rare-about 20 cases per 100,000 individuals -many patients are misdiagnosed as having heart failure or walking pneumonia, or are otherwise not referred soon enough for treatment, notes David Ross, M.D., medical director of the UCLA Lung Transplant Program. "One of the heartbreaking things we see are patients who come to us so late in the course of the disease that they are no longer appropriate transplant candidates," says Dr. Ross. "Early evaluation of patients with pulmonary fibrosis is critical, so that we can assess whether they might benefit from medical therapy, including clinical trials, while also beginning to plan for a possible lung transplant."

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