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.
KEYWORD
Lung transplantation - lymphangioleiomyomatosis - eosinophilic granuloma - histiocytosis
X - sarcoidosis
