CA-125 in Disease Progression and Treatment of Lymphangioleiomyomatosis

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 pConclusions

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