Inverse correlation between VEGF and soluble VEGF receptor 2 in POEMS with AIDP responsive to intravenous immunoglobulin.
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, and skin changes) syndrome is characterized by chronic progressive polyneuropathy and plasma-cell dyscrasia. A major diagnostic criterion of POEMS is elevation of circulating vascular endothelial growth factor (VEGF), which is believed to play a pathogenic role in this disease. We report a case of POEMS that presented as relapsing acute inflammatory demyelinating polyneuropathy, in which complete remission after intravenous immunoglobulin (IVIg) treatment was unexpectedly observed. At clinical nadir, the VEGF level was 30-fold higher, and the soluble form of VEGF receptor 2 (sVEGFR2), which acts as a decoy for VEGF, was 2.7-fold lower than normal. These changes combined might contribute to the pathogenesis of POEMS, inducing vascular permeability and tissue edema. At 9-month follow-up, during clinical remission, VEGF and sVEGFR2 were near normal values. sVEGFR2 reduction is a new finding in POEMS. IVIg treatment may benefit POEMS patients with acute neuropathy by downgrading VEGF release induced by inflammatory cytokines.
Terracciano
Chiara
C
Department of Neurosciences, Tor Vergata University of Rome and IRCCS Fondazione Santa Lucia, Rome, Italy.
Fiore
Stefania
S
Doldo
Elena
E
Manzari
Vittorio
V
Marfia
Girolama Alessandra
GA
Bernardi
Giorgio
G
Massa
Roberto
R
Albonici
Loredana
L
D000200:Action Potentials / Q000502:physiology; D004568:Electrodiagnosis; D004576:Electromyography; D004594:Electrophysiology; D020275:Guillain-Barre Syndrome / Q000150:complications / Q000378:metabolism* / Q000628:therapy*; D006801:Humans; D007124:Immunoenzyme Techniques; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D018482:Muscle, Skeletal / Q000294:innervation / Q000502:physiology; D016878:POEMS Syndrome / Q000150:complications / Q000378:metabolism* / Q000628:therapy*; D010386:Pelvic Neoplasms / Q000150:complications / Q000628:therapy; D010954:Plasmacytoma / Q000150:complications / Q000628:therapy; D011984:Sensory Receptor Cells / Q000502:physiology; D013922:Thrombocytosis / Q000150:complications; D014057:Tomography, X-Ray Computed; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism*; D040301:Vascular Endothelial Growth Factor Receptor-2 / Q000378:metabolism*
D016756:Immunoglobulins, Intravenous; D042461:Vascular Endothelial Growth Factor A; D040301:Vascular Endothelial Growth Factor Receptor-2