[Pulmonary lymphangioleiomyomatosis: an often fortuitous diagnosis. A case report].

Abstract

Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure.
We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest.
Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.