|Title:||Patient characteristics of Lymphangioleiomyomatosis in an Irish population|
|Author(s):||E Lynn L Browne J Morrow A Sahadevan A Kelly A Fabre J.D. Dodd2 M P. Keane D.J. Murphy C McCarthy|
|Institution:||St Vincent's University Hospital/ University College Dublin|
|Poster:||Click to view poster|
|Abstract:||Lymphangioleiomyomatosis (LAM) is a metastasizing neoplasm resulting in parenchymal destruction and cystic lung disease1. It has an estimated prevalence of 3-8 cases per million women.|
Data on patients with LAM attending our cystic lung disease clinic was analysed to characterise this cohort. Age, history of pneumothorax, mode of diagnosis, VEGF-D levels, PFTs, CT cyst scores and mTOR treatment was assessed.
Twenty seven females with LAM were included, 63% diagnosed radiologically, 22% with VEGF-D levels meeting diagnostic criteria and 15% with confirmed tissue diagnosis. Mean age was 50.52 years (range 27–81 ). 93%(n=25) were sporadic LAM, 67% were pre-menopausal. 26% (n=7) were treated with mTOR inhibitors. 30% had a history of pneumothorax, 14% chylothorax and 33% angiomyolipoma. 15% required long term oxygen. Mean VEGF-D level was 626pg/ml (+/-599.48). Mean DLCO and FEV1 were 64.52% (+/-27.35) and 85.7% (+/-16.05) respectively. VEGF-D inversely correlated with both DLCO (R2=0.4468,p=0.0034) and FEV1% (R2=0.3281,p=0.013)
LAM is a rare disease2, however correct diagnosis is important as it has implications for lifestyle modification and there is effective therapy available. With at least 27 patients identified in Ireland, this represents a prevalence of 11.2 per million women, and is likely higher than this both in Ireland and worldwide.