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POEMS syndrome is a multiorgan disorder defined by the association of polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M), and skin changes (S).1 Serum levels of vascular endothelial growth factor (VEGF) are often markedly elevated in patients with POEMS,2-5 but not with other monoclonal gammopathies.2,4,5 Few patients with other neuropathies have been examined,2-5,6 so that the specificity of elevated VEGF levels for POEMS among patients with neuropathy remains unclear, especially when levels are moderately increased.
Methods.
We measured serum VEGF in 161 patients with neuropathy or related syndromes consecutively recruited and tested for antinerve antibodies at our neuropathy clinic. Six patients had POEMS diagnosed in the presence of at least four of the five features of POEMS, 13 had Guillain-Barré syndrome (GBS), 33 had chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with no other feature of POEMS, 13 had multifocal motor neuropathy (MMN), 19 had neuropathy associated with IgM monoclonal gammopathy of undetermined significance (MGUS) (PN+IgM) with anti-MAG or anti-sulfatide IgM, 49 had neuropathies of other (diabetes in 8, multiple myeloma or lymphoma in 8, systemic vasculitis or other rheumatic diseases in 5, amyloidosis in 3, toxic in 2, inherited in 2, paraneoplastic and small fiber neuropathy in 1 each) or of undetermined causes (19) (other PN), and 28 had amyotrophic lateral sclerosis (ALS). We also tested 21 patients with MGUS or multiple myeloma (MM) without neuropathy (MGUS/MM), and 22 …
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