[Pulmonary lymphangioleiomyomatosis--review and personal results]

Pulmonary lymphangioleiomyomatosis (LAM) is characterised by the proliferation of smooth muscle cells in the peribronchial, perivascular and perilymphatic tissue resulting in severe destructive changes of the lung parenchyma. We reviewed the literature and compared the findings in chest radiograph and CT with our own results obtained in a group of 12 patients suffering from histological proven LAM. The main findings of LAM in chest radiograph are a diffuse reticular opacity and an increased lung volume, often large cysts are visible. The CT scans reveal that cystic lesions are present in all patients, but the reported size of the cystic lesions is contradictory. We postulate two main types of LAM: the one resulting in a diffuse distribution of multiple small cysts, partially demonstrating a honey-combing pattern, the other showing severe destruction of the lung parenchyma due to large cysts. The presence of fibrotic changes and architectural distraction does not exclude LAM. So chest radiograph as well as computed tomography reveal neither uniform nor pathognomonic findings. Differential diagnosis of LAM seems to be more difficult than most authors believe.