S.J. Bourke and D.J. Hendrick

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

ISBN: 9780199204854
Published online May 2012 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks


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  • Respiratory Medicine and Pulmonology


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Lymphangioleiomyomatosis is caused by mutations (usually sporadic, sometimes in tuberous sclerosis) of the TSC1 or TSC2 genes and results in cystic destruction of the lungs, with CT features being sufficiently characteristic to establish the diagnosis in many cases. Two-thirds of patients suffer pneumothoraces. Hormonal therapy with progesterone or tamoxifen is usually given for progressive disease; medical or surgical pleurodesis is advisable; lung transplantation is the main option for advanced disease.

Chapter.  833 words.  Illustrated.

Subjects: Respiratory Medicine and Pulmonology

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