Lung Transplantation for Cystic Lung Diseases: Lymphangioleiomyomatosis, Histiocytosis X, and Sarcoidosis

Abstract

Lung transplantation for respiratory failure due to lymphangioleiomyomatosis, histiocytosis X, and sarcoidosis is a therapeutic option in selected patients. These indications account for less than 6% of all lung transplant procedures, and experience is relatively scarce. Pulmonary function testing at evaluation usually shows an obstructive pattern in lymphangioleiomyomatosis, whereas histiocytosis presents with a mixed pattern, and sarcoidosis with a restrictive pattern. Lung diffusion capacity is similarly reduced, however, only histiocytosis and sarcoidosis are often associated with pulmonary hypertension. In all three diseases extrapulmonary manifestations, although of different kinds, are common and must be investigated before transplantation to avoid posttransplant diagnostic pitfalls. In all these diseases, single and bilateral transplantation show comparable results. Typical complications in lymphangioleiomyomatosis include intraoperative severe intrathoracic bleeding, posttransplant chylothorax, pneumothorax, and bleeding of angiomyolipomas. In histiocytosis, extrapulmonary manifestations in bones and pituitary gland may progress. These diseases typically recur in the allograft, leading to clinical symptoms in histiocytosis whereas recurrence in lymphangioleiomyomatosis and sarcoidosis is most often asymptomatic. Survival is similar to patients transplanted for other diseases.