[Lymphangioleiomyomatosis: study of 15 patients]

Lymphangioleiomyomatosis (LAM) is an uncommon disease of not well-known evolution. We describe the clinical features and follow-up of a group of patients diagnosed of LAM in a hospital pulmonary transplantation programme.

15 women (mean age: 43, range: 36-52) diagnosed of LAM, 9 at the Hospital Vall d'Hebron and 6 referred from other hospitals, for preoperative evolution of lung transplantation, were retrospectively studied.

Dyspnea appeared in all cases and it was the main symptom. Pleural problems in the early evolution of the disease were also very frequent (12 out of 15 patients). For this reason, pleurodesis was performed in 7 patients and pleurectomy in 2 (one of them bilateral). Thoracic CT scan showed very characteristic cystic images and abdominal CT proved the presence of extrathoracic associated angiomyolipomas in one case. In two patients, LAM was diagnosed after studying the explanted lung. These two patients had been previously misdiagnosed of emphysema and pulmonary haemosiderosis. Survival since the beginning of symptoms was 82 and 49%, 5 and 10 years later, respectively. Six out of eight patients who underwent lung transplantation had a long postoperative survival.

LAM seems to be as infrequent in Catalunya as in other countries, even if some cases could be misdiagnosed. Lung transplantation is useful in the advanced stages of the disease and, in our experience, it has improved respiratory insufficiency in half of the patients.