Interstitial Lung Diseases Program in NIH Network

UAB Synopsis, Vol. 27, No. 22, June 9, 2008

The UAB Interstitial Lung Diseases Program provides state-of-the-art, comprehensive evaluation and treatment for patients suffering from fibrosing lung diseases.

“Clinicians and basic scientists collaborate to generate new insights into disease mechanisms and participate in multicenter studies of drugs and novel therapeutic modalities,” Joao A. de Andrade, MD, program director and principal investigator, says.

Interstitial lung diseases (ILD) are a heterogeneous group of disorders linked because of a common finding of inflammation and fibrosis in the interstitial space of the lungs. The chronic lung diseases that fall under the ILD umbrella number in the hundreds, each with a distinct clinical course, radio­-graphic pattern, and histology.

The UAB program is 1 of only 12 chosen to participate in the Idiopathic Pulmonary Fibrosis (IPF) Clinical Research Network, which the NIH established in 2005 to advance development of new treatments.

The presentation of most interstitial lung diseases is nonspecific, and the variety of ILDs makes specific diagnosis difficult. “A careful clinical assessment is a necessity. Diagnosis and treatment require dynamic interaction among radiologists, pathologists, and pulmonologists,” Dr. de Andrade says. He and pulmonologist Joseph B. Barney, MD; pathologist C. Bruce Alexander, MD; cardiothoracic surgeon Robert J. Cerfolio, MD; and radiologists Hrudaya Nath, MD, and Colleen Sanders, MD, collaborate to determine each patient’s specific diagnosis.

“Patients who complain of breathlessness with persistent cough often are thought to have chronic obstructive pulmonary disease, heart disease, or gastro-esophageal reflux with bronchospasm, but ILD should be considered,” Dr. de Andrade says.

About 65% of patients at UAB’s Interstitial Lung Disease Clinic have IPF. The number of IPF patients is higher than previously thought, Dr. de Andrade says, with a prevalence of 20 to 40 per 100,000.

Early intervention is imperative. IPF has a 50% mortality rate within 3 to 5 years of diagnosis. Treatment strategies aim to decrease morbidity while improving quality of life through supplemental oxygen, rehabilitation, and eventually palliative care; however, current therapies have shown little or no survival benefit. Patients younger than 65 years without significant comorbidities are candidates for lung transplant, a costly option available to few.

The program’s Web site, www.uab.edu/ILD, contains data on the radiographic appearance and pathology of IPF and links to information about IPF diagnosis and treatment.

The clinic meets twice weekly and sponsors a patient support group that meets quarterly. To refer a patient or inquire about clinical trials, call 205.934 .7557 or 205.934.7630.

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