Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease characterised by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects predominantly females and can occur sporadically or in patients with tuberous sclerosis complex. This review describes the recent progress in our understanding of the molecular pathogenesis of the disease and LAM cell biology. It also summarises current therapeutic approaches and the most promising areas of research for future therapeutic strategies.
Publication types
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Research Support, N.I.H., Intramural
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Review
MeSH terms
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Cell Proliferation*
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Diagnosis, Differential
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Female
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Gene Expression Regulation
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Humans
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Lung / diagnostic imaging
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Lung / metabolism
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Lung / pathology*
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Lymphangioleiomyomatosis / diagnostic imaging
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Lymphangioleiomyomatosis / etiology
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Lymphangioleiomyomatosis / genetics
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Lymphangioleiomyomatosis / metabolism
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Lymphangioleiomyomatosis / pathology*
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Lymphangioleiomyomatosis / therapy
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Magnetic Resonance Imaging
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Myocytes, Smooth Muscle / diagnostic imaging
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Myocytes, Smooth Muscle / metabolism
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Myocytes, Smooth Muscle / pathology*
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Predictive Value of Tests
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Risk Factors
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Sex Factors
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Signal Transduction
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Tomography, X-Ray Computed
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Treatment Outcome
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Tuberous Sclerosis / complications