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Original Research |

CA-125 in Disease Progression and Treatment of Lymphangioleiomyomatosis

Connie G. Glasgow, BA; Gustavo Pacheco-Rodriguez, PhD; Wendy K. Steagall, PhD; Mary E. Haughey, RN, BSN; Patricia A. Julien-Williams, BSN, MSN-CRNP; Mario P. Stylianou, PhD; Bernadette R. Gochuico, MD; Joel Moss, MD, PhD
Author and Funding Information

No conflicts exist for any of the authors.

Funding information: The study was supported by the Intramural Research Program, NIH, NHLBI and NHGRI.

These data have been presented in abstract form at the International Conference of the American Thoracic Society, 2016, San Francisco, CA.

1Cardiovascular and Pulmonary Branch

2Office of the Clinical Director

3Office of Biostatistics Research, National Heart, Lung, and Blood Institute

4Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland

Corresponding author: Joel Moss, M.D., Ph. D. Cardiovascular and Pulmonary Branch National Heart, Lung, and Blood Institute National Institutes of Health Bldg. 10/Room 6D05/MSC 1590 Bethesda, MD 20892-1590.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.05.018
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Abstract

Background  Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor suppressor genes. Based on case reports, CA-125 (an ovarian cancer biomarker) can be elevated in LAM patients. We hypothesized that elevated serum CA-125 levels seen in some LAM patients were due to LAM, not other malignancies, and might respond to sirolimus treatment.

Methods  Serum CA-125 levels were measured for 241 patients at each visit. Medical records were reviewed for co-morbidities, disease progression, and response to sirolimus treatment. CA-125 expression in LAM cells was determined by immunohistochemistry.

Results  Almost 25 percent of LAM patients had at least one elevated serum CA-125 measurement. Higher serum CA-125 levels correlated with lower FEV1, premenopausal status, and pleural effusion in a multivariate model (each p<0.001). Serum CA-125 levels decreased after sirolimus treatment (p=0.002). CA-125 and alpha smooth muscle actin were co-expressed in LAM lung nodules.

Conclusions  Higher serum CA-125 levels are associated with pleural effusions and reduced pulmonary function and are decreased by sirolimus therapy. LAM cells express CA-125. Some elevated serum CA-125 levels may reflect serosal membrane involvement.


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