In order to determine if these clinical guidelines and biomarkers can improve clinical care for LAM patients by reducing the need for invasive diagnostic investigations and making more accurate predictions of prognosis, we have compared the clinical utility of serum VEGF-D, MMP-2, MMP-9 and ACE with the recent ERS diagnostic guidelines, health-related quality of life and clinical phenotype in a national cohort of patients with LAM. This evidence concerns the gene ACE and lymphangioleiomyomatosis.