Molecular Evidence

  • Vascular endothelial growth factor (VEGF) is an signal protein that promotes cell survival, angiogenesis, and vascular permeability. VEGF serum levels are often elevated in parallel with iMCD symptoms. 
    • Elevated VEGF could account for the hypervascularization in lymph nodes, extravascular fluid overload, and eruptive cherry haemangiomatosis observed in iMCD patients.
  • Elevated systemic VEGF levels typically decrease in iMCD cases following anti-IL-6 treatment, suggesting that VEGF release is downstream of IL-6.
    • However, VEGF’s role has not been fully elucidated in iMCD, and it may be upstream of IL-6 in some patients as it is in the related disease POEMS syndrome.
Clinical Evidence

  • VEGF levels were reported for 20 patients and elevated in 16 (18%). 
  • Concentrations were frequently elevated in patients from past studies, but the reason for this is unknown. 
  • Increased levels of VEGF along with soluble IL-2 receptor were earliest indicators of impending relapse and failure of anti-IL-6 therapy in one patient.