Lymphangioleiomyomatosis (LAM ) is a rare disease characterized by morphofunctional alterations and caused by the proliferation of immature smooth muscle cells within different organs. Lungs or other extra-thoracic organs such as kidney, liver, and intestine, may be involved.
The Lymphangioleiomyomatosis affects women, especially in childbearing age. It represents about 1 % of the interstitial pulmonary diseases. The most affected age group is between the third and fourth decade of life. Recently, an histologically confirmed case in an individual male has been reported. Currently there is no curative treatment for this disease. Nowadays some benefits have been obtained by administration of progestin hormone that seems to slow down the progression of the disease. However, the role of hormonal therapy in the development of LAM, is still under investigation. Some manifestations of this disease are so serious that lung transplantation is required. Although disease recurrences have been described in some cases of lung transplantation, the rarity of this occurrence does not justify a revision of the indication for transplantation. Sometimes, the LAM is associated with Tuberous Sclerosis and this association, which suggests a genetic predisposition, is matter of the research activity.
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Fondazione Malattie Rare “Mauro Baschirotto” Onlus
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