| Title: | Mode of Presentation, Diagnosis and Prevalence of Lymphangioleiomyomatosis in an Irish population |
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| Author(s): | E Lynn M O’Callaghan L Browne M.P. Keane C McCarthy |
| Institution: | St. Vincent's University Hospital |
| Poster: | Click to view poster |
| Category: | ILD |
| Abstract: | Lymphangioleiomyomatosis (LAM) is a low-grade metastasizing neoplasm causing cystic lung disease.1 It impacts women during reproductive years. Estimated prevalence is 3-8 cases per million.2 Characteristic HRCT Thorax plus; serum VEGF-D ≥800pg/ml, TSC, angiomyolipomas (AML), chylous effusions or lymphangioleiomyomas are diagnostic. Data on 36 LAM patients was analysed to establish initial mode of presentation and classify the findings used to confirm diagnosis. 52% (n=19) presented with shortness of breath, 5% (n=2) with chest pain and 25% (n=9) had a pneumothorax. 17% (n=6) had an incidental diagnosis based on CT imaging for other conditions. 67% of patients were pre-menopausal; 11% (n=4) were peri-partum at initial presentation. 25% (n = 9) had evidence of an AML, 5% (n=2) had TSC, 19% (n=7) had VEGF-D levels >800pg/ml, 16% (n=6) had chylothorax and 19% (n=7) of patients have histopathology confirmation of LAM. Our data suggests the prevalence of LAM to be 14.5 per million women in Ireland, higher than that reported worldwide. While the majority of patients presented symptomatically, 17% had an incidental diagnosis highlighting the need for clinicians to consider LAM in patients with cystic lung disease. |
