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Measurements of serum vascular endothelial growth factor (VEGF) could prevent misdiagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome
POEMS syndrome is a rare cause of demyelinating polyneuropathy with multiorgan involvement.1 The prevalence of the disorder was reported to be 0.3 per 100 000 population in Japanese nationwide survey,2 whereas it may be lower in Western countries. In patients with typical POEMS features, the diagnosis is relatively easy, but up to 50% of patients initially develop demyelinating polyneuropathy alone.2
Separately, chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic immune-mediated demyelinating neuropathy; the reported prevalence ranges from 0.8 to 8.9 per 100 000 people.3 The diagnosis of CIDP is based on electrodiagnostic evidence of peripheral nerve demyelination, and exclusion criteria. Both patients with POEMS and CIDP show symmetric demyelinating polyneuropathy, and previous studies have shown that up to 60% of patients with POEMS syndrome were initially diagnosed as having CIDP by neurologists; specifically, actually 70% of patients with POEMS fulfilled the electrodiagnostic criteria for definite CIDP.4 The fact is very critical in clinical practice; many patients with POEMS syndrome are misdiagnosed with CIDP, and …
Footnotes
Contributors SK wrote the manuscript. TS and SM made revisions.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; externally peer reviewed.