ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is typically a fatal disease that fails to respond to medical therapy. For these patients lung transplantation offers the promise of improved quality and duration of life. The initial reports of successful transplantation for IPF date back to the mid-1980s although recent data suggest IPF patients make up nearly 20% of all single lung transplants. The survival rates following lung transplantation for IPF are estimated at 67% for 1 year, 52% for 3 years, and 35% for 5 years. Mortality rates following lung transplantation for IPF are higher than emphysema, but are generally comparable to primary pulmonary hypertension. Given the relatively high mortality following transplant the decision of when to transplant a patient is of paramount importance. Although individual patients differ, generalizations predicting a poor prognosis include the diagnosis of usual interstitial pneumonia (UIP), a forced vital capacity or total lung capacity of less than 65% predicted, a diffusion capacity for carbon monoxide (DLCO) less than 45% of predicted, and the presence of extensive honeycombing on high-resolution computed tomographic scans. The presence of any of these features should prompt the patient and physician to consider lung transplantation as a potential therapeutic modality. Patients with interstitial lung disease related to collagen vascular diseases or other causes need to be considered on an individual basis; their prognosis is usually better than patients with IPF, and the potential for systemic involvement may preclude listing for lung transplantation.
KEYWORD
Idiopathic pulmonary fibrosis - cryptogenic fibrosing alveolitis - interstitial lung disease - collagen vascular disease - nonspecific interstitial pneumonia - lung transplantation
