Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex

The true prevalence of pulmonary lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex (TSC) is unknown. The prevalence of LAM, radiological features, and lung function in patients with TSC was measured. The presence of LAM, as defined by the presence of cysts by high-resolution...

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Bibliographic Details
Published in:American journal of respiratory and critical care medicine 2001-08, Vol.164 (4), p.669-671
Main Authors: MOSS, Joel, AVILA, Nilo A, BARNES, Pauline M, LITZENBERGER, Ruth A, BECHTLE, Jeanmarie, BROOKS, Pamela G, HEDIN, Carolyn J, HUNSBERGER, Sally, KRISTOF, Arnold S
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Forced Expiratory Volume
Humans
Lung Neoplasms - diagnostic imaging
Lung Neoplasms - epidemiology
Lung Neoplasms - genetics
Lung Neoplasms - physiopathology
Lymphangioleiomyomatosis - diagnostic imaging
Lymphangioleiomyomatosis - epidemiology
Lymphangioleiomyomatosis - genetics
Lymphangioleiomyomatosis - physiopathology
Mass Screening
Medical sciences
Neurology
Population Surveillance
Prevalence
Prospective Studies
Registries
Respiratory Function Tests
Severity of Illness Index
Smoking - adverse effects
Tomography, X-Ray Computed
Tuberous Sclerosis - complications
Tumors of the nervous system. Phacomatoses
United States - epidemiology
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Summary:The true prevalence of pulmonary lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex (TSC) is unknown. The prevalence of LAM, radiological features, and lung function in patients with TSC was measured. The presence of LAM, as defined by the presence of cysts by high-resolution chest computed tomography (HRCT) scan, was determined in patients with TSC without prior pulmonary disease (Group 1). To determine the significance of early detection, severity of disease in screened patients (Group 1) was compared with that in patients with TSC with a prior diagnosis of LAM (Group 2). Forty-eight patients with TSC and no prior history of LAM were screened. Of the 38 females, 13 (34%) had LAM; LAM was absent in males. Lung function was preserved in patients with TSC who were found to have LAM by screening. In patients previously known to have LAM, FEV(1) and DL(CO) correlated inversely with severity of disease as assessed by CT scan. The prevalence of LAM in women with TSC was 34%, approximately 10-fold that previously reported, consistent with a large hitherto unrecognized subclinical population of patients at risk for pulmonary complications.
ISSN:1073-449X
1535-4970
DOI:10.1164/ajrccm.164.4.2101154