POEMS syndrome with multiple ganglioside/paraneoplastic antibodies misdiagnosed as CIDP: a case report - Report - MDSpire

POEMS syndrome with multiple ganglioside/paraneoplastic antibodies misdiagnosed as CIDP: a case report

  • By

  • Mengmeng Fan

  • Jiaxiu He

  • Shihui Sun

  • Linyuan He

  • Zhao Zhong

  • April 30, 2026

  • 0 min

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Clinical Report: Misdiagnosis of POEMS Syndrome as CIDP

Overview

This report highlights a case of POEMS syndrome misdiagnosed as CIDP, emphasizing the importance of distinguishing between these conditions. The findings underscore the need for heightened clinical awareness and appropriate diagnostic evaluations to avoid misdiagnosis.

Background

POEMS syndrome is a rare disorder with significant systemic involvement, often leading to misdiagnosis as CIDP due to overlapping symptoms. Accurate differentiation is crucial, as the management and treatment strategies for these conditions differ markedly. Understanding the clinical and laboratory features of POEMS is essential for timely diagnosis and intervention.

Data Highlights

No numerical data or trial data presented in the article.

Key Findings

  • POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein secretion, and skin changes.
  • Initial symptoms in the reported case included lower limb paresthesia, which progressed over seven months.
  • Electromyography revealed peripheral neuropathy with demyelinating lesions and axonal damage.
  • Laboratory findings included a monoclonal IgA-λ band, indicating plasma cell dyscrasia.
  • Misdiagnosis of POEMS as CIDP can lead to inappropriate treatment and delayed management.
  • Clinical vigilance and comprehensive diagnostic evaluations are necessary to differentiate between POEMS and CIDP.

Clinical Implications

Healthcare professionals should maintain a high index of suspicion for POEMS syndrome in patients presenting with neuropathy and systemic symptoms. Early identification and appropriate diagnostic workup, including serum VEGF levels and hematologic evaluations, are critical for effective management.

Conclusion

The case underscores the importance of distinguishing POEMS syndrome from CIDP to ensure accurate diagnosis and treatment. Enhanced clinical awareness and diagnostic rigor are essential in managing these complex conditions.

References

  1. Syed Ali Abutalib, MD Angela Dispenzieri, MD, ASCO Post, 2026 -- POEMS Syndrome: Diagnostic Clues From Neuropathy to Bone Marrow Findings
  2. Clinical Rheumatology, 2026 -- Paraneoplastic dermatomyositis with atypical features associated with a solid pseudopapillary pancreatic neoplasm
  3. Acta Neuropathologica, 2020 -- Pathological Insights into Inflammatory Demyelinating Disorders of the Central Nervous System Associated with Myelin Oligodendrocyte Glycoprotein Autoantibodies
  4. EAN/PNS Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Guideline Summary - Guideline Central
  5. Acta Neuropathologica — Teratoma Expressing MOG Preceding Optic Neuritis Associated with MOG-IgG
  6. EAN/PNS Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Guideline Summary - Guideline Central
  7. POEMS syndrome: diagnosis, treatments, and outcomes - PMC
  8. Efgartigimod as a treatment for people with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a plain language summary of publication of the ADHERE trial - PMC

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