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Related Experiment Video

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Nerve pathologic features differentiate POEMS syndrome from CIDP.

Ezequiel A Piccione1, Janean Engelstad2, Peter J Dyck2

  • 1University of Nebraska Medical Center, 988435 Nebraska Medical Center, Omaha, NE, 68198-8435, USA.

Acta Neuropathologica Communications
|November 2, 2016
PubMed
Summary

Nerve pathology differs between POEMS syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). POEMS shows more axonal damage and vessel growth, while CIDP exhibits greater inflammation and onion-bulb formations, aiding diagnosis.

Keywords:
CIDPDemyelinationNeovascularizationPOEMSVEGF

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Area of Science:

  • Neurology
  • Pathology
  • Immunology

Background:

  • POEMS syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP) are distinct neuropathies.
  • Differentiating these conditions is crucial for appropriate treatment.
  • Nerve biopsy findings may offer diagnostic insights.

Purpose of the Study:

  • To compare nerve pathology in POEMS syndrome versus CIDP.
  • To identify distinct histological features differentiating the two conditions.
  • To test the hypothesis that POEMS involves more vascular and axonal changes, and less inflammation than CIDP.

Main Methods:

  • Retrospective analysis of 61 nerve biopsies (35 POEMS, 26 CIDP).
  • Blinded review of teased fibers, paraffin sections, semithin sections, and immunostains.
  • Quantification of small endoneurial and epineurial vessels using smooth muscle actin staining.

Main Results:

  • POEMS group showed significantly higher axonal degeneration and fewer normal myelinated fibers.
  • CIDP group exhibited significantly more endoneurial mononuclear inflammation and large onion-bulb formations.
  • POEMS biopsies had more epineurial vessels; 120 vessels predicted differentiation (77% specific, 54% sensitive).

Conclusions:

  • Nerve biopsy aids in distinguishing POEMS syndrome from CIDP.
  • POEMS syndrome is characterized by axonal degeneration and epineurial neovascularization.
  • CIDP shows endoneurial inflammation and onion-bulb formation, suggesting different pathogenic mechanisms.