Abstract | BACKGROUND:
Lymphangioleiomyomatosis is a rare disease occurring almost exclusively in women. Diagnosis often requires surgical biopsy and the clinical course varies between patients with no predictors of progression. We evaluated recent diagnostic guidelines, clinical features and serum biomarkers as diagnostic and prognostic tools. METHODS: RESULTS: 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. CONCLUSIONS:
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Authors | William Y C Chang, Jennifer L Cane, John D Blakey, Maruti Kumaran, Kate S Pointon, Simon R Johnson |
Journal | Respiratory research
(Respir Res)
Vol. 13
Pg. 34
(Apr 18 2012)
ISSN: 1465-993X [Electronic] England |
PMID | 22513045
(Publication Type: Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Biomarkers
- Vascular Endothelial Growth Factor D
- Peptidyl-Dipeptidase A
- MMP2 protein, human
- Matrix Metalloproteinase 2
- Matrix Metalloproteinase 9
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Topics |
- Adult
- Biomarkers
(blood)
- Cohort Studies
- Female
- Humans
- Lymphangioleiomyomatosis
(blood, diagnosis)
- Matrix Metalloproteinase 2
(blood)
- Matrix Metalloproteinase 9
(blood)
- Middle Aged
- Peptidyl-Dipeptidase A
(blood)
- Practice Guidelines as Topic
(standards)
- Respiratory Function Tests
(standards)
- Vascular Endothelial Growth Factor D
(blood)
- Young Adult
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