Hi Everyone, I am working with a Dr. Charles Kirkpatrick at the University of Denver Hospital/Anchutz Medical Center to get a final diagnosis before starting IVIG.  I am so thankful to Arturo for his guidance in seeking an immunologist or hemotologist associated with a university hospital.  Dr K is a leading immunologist & occasionally teaches at NIH & knows Dr. Druey. Upon my appointment with Dr. K last month, one of the 14 tests he ran on me was regarding the Vascular Endothelial Growth Factor.  I learned from him today that normal is 0-80, & mine was 170.  He said there are fairly new findings that people with SCLS also have abnormal VEGF.   He is pretty convinced because of my test result that I indeed have been diagnosed correctly with SCLS.  He also wants to rule out any cardiac involvement since 4 of my 5 episodes have occurred during extreme physical exertion.  Therefore, I am scheduled to have an exercise stress test.  Once those results are in and ruled out he plans to set me up for IVIG.   Has anyone been tested & found to have abnormal VEGF?   Thank you. Sincerely, Ann Fallentine af8612

Dear Ann: I'm so happy to hear that you have found a senior doctor with a scientific background that enables him to understand what goes on in a patient with SCLS, and therefore to help you confirm your diagnosis or point you in some other direction! With regard to your question about VEGF, Dr. Druey's research (as published in the article titled "Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease") showed that "In our cohort of 20 patients with classic acute SCLS, VEGF and Ang2 levels were significantly higher than healthy controls without SCLS, which could account for the disrupted adherens junctions observed. In agreement with individual case reports, our patients had elevated serum VEGF levels at the onset of an acute SCLS episode compared with their baseline, and in one patient VEGF returned to baseline rapidly on presentation with a severe hypotensive crisis." Namely, he found a rise in VEGF levels at the start of episodes -- but not elevated levels on an ongoing basis. VEGF is a protein capable of inducing rapid leakage from blood vessels, so Dr. Druey suspects that its elevation (for unknown reasons) may be a cause or contributing factor to why we experience capillary leaks. In sum, if you were found to have a high VEGF when you weren't experiencing an episode of SCLS, then maybe you have some other condition. Your doctor will probably want to measure your VEGF levels several times to see if the high number he measured once was a fluke result.

Thank you Arturo. This is certainly an interesting but a bit overwhelming process. I also inquired of the doctor by email as to whether I was tested for MGUS. Also, I believe he said he had discussed my case with Dr. Druey. I will keep you & the community posted as we progress toward a proper determination. Ann