Long-Term Survival in a Patient with Pulmonary Lymphangioleiomyomatosis

A patient with pulmonary lymphangioleiomyomatosis was diagnosed more than 22 years after the onset of symptoms by a thoracoscopic lung biopsy, after a high resolution computerized tomogram of the chest was highly suggestive of the disease. After nearly 30 years since the onset of her symptoms, the p...

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Bibliographic Details
Main Authors: John K Reid, Henrike Rees, Donald Cockcroft
Format: Article
Language:English
Published: Wiley 2002-01-01
Series:Canadian Respiratory Journal
Online Access:http://dx.doi.org/10.1155/2002/958921
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Summary:A patient with pulmonary lymphangioleiomyomatosis was diagnosed more than 22 years after the onset of symptoms by a thoracoscopic lung biopsy, after a high resolution computerized tomogram of the chest was highly suggestive of the disease. After nearly 30 years since the onset of her symptoms, the patient leads a relatively normal life with only mildly abnormal lung function and has minimal reduction in her exercise tolerance. There have been few reports of patients surviving for such a long time after the onset of this disease; the literature suggests that most patients die within 15 years of symptom onset.
ISSN:1198-2241