%0 Journal Article
%T Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis
%A William YC Chang
%A Jennifer L Cane
%A John D Blakey
%A Maruti Kumaran
%A Kate S Pointon
%A Simon R Johnson
%J Respiratory Research
%D 2012
%I BioMed Central
%R 10.1186/1465-9921-13-34
%X Serum vascular endothelial growth factor-D (VEGF-D), angiotensin converting enzyme (ACE), matrix metalloproteinases (MMP) -2 and -9, clinical phenotype, thoracic and abdominal computerised tomography, lung function and quality of life were examined in a cohort of 58 patients. 32 healthy female controls had serum biomarkers measured.Serum VEGF-D, ACE and total MMP-2 levels were elevated in patients. VEGF-D was the strongest discriminator between patients and controls (median£¿=£¿1174 vs. 332£¿pg/ml p£¿<£¿0.0001 with an area under the receiver operating characteristic curve of 0.967, 95% CI 0.93-1.01). Application of European Respiratory Society criteria allowed a definite diagnosis without biopsy in 69%. Adding VEGF-D measurement to ERS criteria further reduced the need for biopsy by 10%. VEGF-D was associated with lymphatic involvement (p£¿=£¿0.017) but not the presence of angiomyolipomas.Combining ERS criteria and serum VEGF-D reduces the need for lung biopsy in LAM. VEGF-D was associated with lymphatic disease but not lung function.
%K VEGF-D
%K Matrix metalloproteinase
%K Angiotensin converting enzyme
%K ERS LAM guidelines
%U http://respiratory-research.com/content/13/1/34/abstract