To the Editor: Lymphangioleiomyomatosis, a rare lung diseasetypically affecting women of reproductive age, is characterizedby an abnormal proliferation of smooth-muscle cells and theprogressive loss of pulmonary function due to destruction oflung parenchyma.1 Long-term survival is rare; in patients withlymphangioleiomyomatosis, respiratory failure often developswithin 10 years after diagnosis. Currently, there is no standard-of-caretherapy for this disease, although limited success has beenreported with hormone therapy or lung transplantation.
The destruction of lung parenchyma has been attributed to anenvironment conducive to proteolysis resulting from increasedexpression and activity of matrix metalloproteinases (MMPs).MMP-2, in . . . [Full Text of this Article]
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