SESSION TITLE: Pulmonary Manifestations of Systemic Disease Posters
SESSION TYPE: Original Investigation Poster
PRESENTED ON: Wednesday, October 28, 2015 at 01:30 PM - 02:30 PM
PURPOSE: Vascular endothelial growth factor-D (VEGF-D) is a diagnostic marker for lymphangioleiomyomatosis (LAM). An optimal concentration > 800 pg/mL has been used as a conservative threshold for a definitive diagnosis of LAM in patients with typical thin-walled cystic change on chest CT, sensitivity 73% and specificity100%. Our study aimed to evaluate the frequency of elevated level of VEGF-D > 800 pg/ml in patients with LAM and other cystic lung diseases and compare serum VEGF-D level between LAM and other cystic lung disease.
METHODS: We retrospectively reviewed the medical records of patients with cystic lung diseases referred to the LAM Clinic, Mayo Clinic Florida from January 2000 to December 2015. The demographical, clinical, radiological data, pulmonary function test and serum VEGF-D level were collected and analyzed.
RESULTS: There were 33 patients with LAM diagnosis, 30 with sporadic LAM and 3 with tuberous sclerosis complex (TSC)-LAM. All patients were female with the mean age of 53±13 (29-88) years. Sixteen (48%) patients had biopsy-proven LAM and 17 patients had clinically defined LAM. They had moderate obstruction (mean± SD post bronchodilator FEV1 1.87±0.76, 71±23%). The TLC, RV, DLCO were 101±15%, 136±68% and 61±24% respectively. Serum VEGF-D levels were available in 14 of 33 patients all of which had sporadic LAM: 665±689 pg/mL (range 250-2873 pg/mL). Only 2 patients had elevated serum VEGF-D > 800 pg/mL. The mean serum VEGF-D levels of patients with sporadic LAM were significantly higher compared to levels in those with other cystic lung disease (665±689 vs. 239±36, p=0.012) but only 3 patients with other cystic lung disease (2 lymphangiomatosis, 1 unclassified disease) had VEGF-D measured Other patients with cystic lung disease without VEGF-D levels included Birt-Hogg-Dubé syndrome (n=3), lymphocytic interstitial pneumonia (n=2), pulmonary Langerhans cell histiocytosis (n=2), lymphangiomatosis (n=2) and unclassified cystic lung disease (n=5).
CONCLUSIONS: Our single center cohort revealed that only 6% of sporadic LAM had serum VEGF-D concentrations >800 pg/mL which was lower than the published data in the MILES trial. The average serum level of VEGF-D for the patients with sporadic LAM was higher than those with other cystic lung disease; however, the series is small.
CLINICAL IMPLICATIONS: The currently recommended threshold for serum VEGF-D as a diagnostic test to distinguish sporadic LAM from other cystic lung disease may be too conservative.
DISCLOSURE: The following authors have nothing to disclose: Kamonpun Ussavarungsi, Charles Burger
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