Published in UAB Insight, Spring 2008
Evaluation, Treatment of Interstitial Lung Diseases
Interstitial lung diseases (ILD) are a heterogeneous group of disorders grouped together because of a common finding of inflammation and fibrosis in the interstitial spaces of the lungs. The chronic lung diseases that fall under the ILD umbrella number in the hundreds, each with a distinct clinical course, radiographic pattern, and histology.
The UAB Interstitial Lung Diseases Program provides state-of-the-art, comprehensive evaluation and treatment for patients suffering from these conditions. Clinicians and basic scientists collaborate to generate new insights into disease mechanisms and participate in multicenter studies of drugs and novel therapeutic modalities. The program is 1 of only 12 chosen to participate in the Idiopathic Pulmonary Fibrosis (IPF) Clinical Research Network, which the National Institutes of Health established in 2005 to advance development of new treatments. Program Director Joao A. de Andrade, MD, and colleagues host a weekly teaching ILD conference. The clinic meets twice weekly and sponsors a patient support group that meets quarterly.
The presentation of most ILD is nonspecific and the diagnostic key is a high degree of suspicion combined with systematic testing and pathological specificity. A careful clinical assessment may unveil a history of occupational and environmental exposures or underlying autoimmune disorders. “Patients who complain of breathlessness with persistent cough often are thought to have chronic obstructive pulmonary disease, heart disease, or gastroesophageal reflux with bronchospasm, but ILD should be considered,” de Andrade says.
Pulmonary function testing and a high-resolution computerized tomography (HRCT) scan can determine if ILD is a factor, but the variety of ILDs makes specific diagnosis difficult. “When clinicians do find evidence of ILD, they should refer their patient to a specialized center,” de Andrade says. “Diagnosis and treatment require dynamic interaction among radiologists, pathologists, and pulmonologists.”
Idiopathic Pulmonary Fibrosis
About 65% of patients at UAB’s Interstitial Lung Disease Clinic have IPF. The number of IPF patients is higher than previously thought, de Andrade says, with a prevalence of 20 to 40 per 100,000. IPF is a diagnosis of exclusion that can be complex, but radiologists often can identify its distinct pattern on a HRCT scan, with a definitive diagnosis determined by surgical lung biopsy.
Early intervention is imperative. IPF has a 50% mortality rate within 3 to 5 years of diagnosis. “Primary care physicians and pulmonologists often do not refer patients in time for rehabilitation or transplantation to be effective, viable options,” de Andrade says. Patients younger than 65 years without significant comorbidities are candidates for lung transplant, but it is a costly option only available to a select few, with a 5-year survival rate of 50% to 60%.
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. Until evidence-based results show an advantage, patients should be referred to specialty tertiary centers such as UAB for consideration for enrollment in clinical studies.
For more information:
Dr. Joao de Andrade
1.800.UAB.MIST
mist@uabmc.edu