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In vitro studies of lymphangioleiomyomatosis

¹ Dept of Pharmacology, and ² Woolcock Institute of Medical Research, University of Sydney, and ³ Transplant Unit, St Vincents Hospital Darlinghurst, Sydney, Australia.

CORRESPONDENCE: J. L. Black, Dept of Pharmacology, University of Sydney, NSW 2006, Australia. Fax: 61 290365126. E-mail: judblack{at}med.usyd.edu.au

Keywords: Airway smooth muscle, asthma, lymphangioleiomyomatosis, matrix proteins

Received: February 14, 2005
Accepted May 15, 2005

Lymphangioleiomyomatosis (LAM) is associated with abnormal airway smooth muscle that leads to the characteristic pathology of lung nodule formation and destruction of lung tissue. The current authors have previously identified abnormal behaviour of airway smooth muscle cells from patients with asthma.

In this study, cells and tissue sections derived from patients with LAM (n = 7), asthma (n = 8), and nonasthmatic controls (n = 9) were compared. The presence of the antigen human melanosome (HM)B-45 was investigated, along with the proliferation and release of extracellular matrix proteins, release of endogenous prostaglandin E₂ (PGE₂), vascular endothelial growth factor and connective tissue growth factor, and the expression of integrins.

Positive HMB-45 staining was found in all LAM patients and no controls. Proliferation of LAM cells was not different from control cells nor was its inhibition by ß-agonists, corticosteroids, rapamycin or PGE₂. However, endogenous PGE₂ levels were markedly decreased in LAM cells, and this was associated with decreased expression of the inducible form of cyclooxygenase (COX-2). The increased levels of connective tissue growth factor seen in asthma cells were not observed in LAM. Elastin mRNA in response to transforming growth factor-ß stimulation was markedly lower in LAM cells than either asthma or control cells.

In conclusion, lymphangioleiomyomatosis cells exhibit abnormal properties in vitro that may contribute to pathophysiology and symptomatology in patients with lymphangioleiomyomatosis.