Angiomyolipomas are Indicator Lesions for Sporadic Lymphangioleiomyomatosis in Women

摘要

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease, almost exclusively affecting women, that can occur sporadically or in association with tuberous sclerosis. It is caused by proliferating smooth muscle-like cells. Symptoms of LAM are dyspnoea, pneumothorax, chylothorax, and haemoptysis. Life expectancy of women affected by LAM is assumed to be between 10 and 20 years from diagnosis. So far, no causal therapy for LAM exists. Recent data indicate that rapamycine and doxycy-cline might be of therapeutic benefit (for a review, see Goncharova and Krymskaya ) and that LAM cells express various markers that are currently targeted by innovative therapeutic strategies such as kinase inhibitors and antibody-based therapy .