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Inverse correlation between VEGF and soluble VEGF receptor 2 in POEMS with AIDP responsive to intravenous immunoglobulin
Author(s) -
Terracciano Chiara,
Fiore Stefania,
Doldo Elena,
Manzari Vittorio,
Marfia Girolama Alessandra,
Bernardi Giorgio,
Massa Roberto,
Albonici Loredana
Publication year - 2010
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21718
Subject(s) - poems syndrome , organomegaly , polyneuropathy , medicine , plasma cell dyscrasia , vascular endothelial growth factor , gastroenterology , pathogenesis , edema , vascular permeability , chronic inflammatory demyelinating polyneuropathy , antibody , pathology , immunology , vegf receptors , immunoglobulin light chain
Abstract 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. Muscle Nerve, 2010