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Minerva Pneumologica 2008 June;47(2):63-72

Copyright © 2008 EDIZIONI MINERVA MEDICA

language: English

Lung transplantation in sarcoidosis

Morgenthau A. S., Michael C., Lannuzzi M.

Department of Medicine Division of Pulmonary, Critical Care and Sleep Medicine Mount Sinai School of Medicine New York, NY, USA

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Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Greater than 90% of sarcoidosis patients demonstrate lung disease. Corticosteriods alleviate symptoms in most patients but approximately 30% of patients develop chronic, progressive disease. End-stage pulmonary fibrosis is the most frequent cause of death. Lung transplantation is a treatment option for sarcoidosis patients with end-stage fibrosis but it should only be considered when medical therapy has been exhausted. Enactment of the Lung Allocation Score system has changed allocation from a system based purely on waiting time to one that attempts to balance waiting time against survival probability on the waiting list and after transplantation. Mortality for sarcoidosis patients listed for lung transplantation is most closely linked to elevated right atrial pressure. Patients with comorbid pulmonary hypertension should promptly be referred for lung transplantation. African American patients with sarcoidosis appear to have a more than two-fold increased risk of death after lung transplantation. Sarcoidosis may recur after lung transplantation. Patients with recurrent sarcoidosis rarely exhibit pulmonary symptoms or radiologic changes that are consistent with the disease. Fluorescence in situ hybridization techniques suggest that recurrent granulomas in the transplanted lung are derived from recipient’s immune cells. Further studies are needed to elucidate the immunologic mechanisms, which mediate recurrent sarcoidosis.