Pulmonary lymphangioleiomyomatosis: fortuitous diagnosis.

Introduction. - Lymphangioleiomyomatosis is an Imcornmon disorder of unknow n origin, which exclusively occurs in women of reproductive age. The condit ion is characterized by proliferation of immature smooth muscle cells throu ghout the lungs, i.e., in the peribronchial, perilymphatic, and perivascula r areas. This results in obliteration of the respiratory tract a,td in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respirat ory failure. Exegesis. - We report the case of a patient in whom lymphangioleiomyomatosi s was fortuitously diagnosed from chess CT scan, itself performed for the d iagnosis of pulmonary embolism. This case is therefore of particular intere st. Conclusion. - Our results suggest that the prevalence of lymphangioleiomato sis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive ra diological method's should permit early diagnosis of the disease. (C) 1999 Elsevier, Paris.