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Vital Signs

Spring 2005

UCLA Tests Novel Approaches to Treat Lung Disease

Commonly misdiagnosed, idiopathic pulmonary fibrosis is a rare, yet devastating, lung disease

New approaches to treating a rare, yet devastating, lung disease are being tested through UCLA’s Interstitial Lung Disease Clinical Program. Idiopathic pulmonary fibrosis (IPF)—characterized by progressive shortness of breath, exercise limitations, the need for oxygen, and progressively worsening lung function—mainly affects people in their mid-70s, yet can occur in younger people.

IPF is one of 150 interstitial diseases, some of which have symptoms that mimic IPF and for which effective treatments are available.

“Misdiagnoses are common. Many people may have erroneously been told they have bronchitis, emphysema or walking pneumonia,” notes Joseph Lynch III, M.D., UCLA pulmonologist. A majority of patients with IPF die within three years of diagnosis; more than 70 percent die within eight years from the onset of symptoms.

Until now, few studies with scientific controls have been used to explore the usefulness of various drugs on the disease. Steroids have often been prescribed as have immunosuppressant drugs, but Dr. Lynch warns that they probably cause more harm than benefit. Among other studies, UCLA is participating in a clinical trial to treat patients with mild IPF disease using gamma interferon, a man-made version of a substance naturally produced by cells in the body to fight infections and tumors. Preliminary data suggest there may be some benefit in patients with less severe disease. In addition, under the leadership of David Zisman, M.D., UCLA pulmonologist and critical care specialist, a variety of additional pharmaceutical agents with antifibrotic and angiostatic properties are being investigated to treat IPF.

“A correct diagnosis is essential for appropriate treatment,” Dr. Lynch stresses. Diagnostic tools include computerized tomography (CT) scans of the chest and lung biopsy.

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