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Severe CIDP-MGUS responsive to Rituximab.

Andreas Posa1, Alexander Emmer1, Malte Kornhuber1

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Heliyon
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Summary

Chronic inflammatory demyelinating polyneuropathy with monoclonal gammopathy of undetermined significance (CIDP-MGUS) is a rare CIDP variant. B cell depletion therapy using rituximab showed positive clinical and electrodiagnostic effects in a CIDP-MGUS patient.

Keywords:
AnatomyB-cell dyscrasiaChronic Inflammatory Demyelinating Polyneuropathy (CIDP)Monoclonal Gammopathy of Undetermined Significance (MGUS)Musculoskeletal systemNerve Conduction Sudies (NCS)Nervous systemNeurologyPhysiologyRituximab

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Area of Science:

  • Neurology
  • Immunology

Background:

  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder causing progressive limb weakness and sensory loss.
  • Some CIDP cases exhibit severe progression unresponsive to standard therapies like intravenous immunoglobulin or plasma exchange.
  • CIDP with monoclonal gammopathy of undetermined significance (CIDP-MGUS) is a rare variant with limited therapeutic research.

Observation:

  • A specific case of CIDP-MGUS presented with a severe, treatment-resistant disease course.
  • The patient's condition was monitored through clinical assessments and nerve conduction studies.

Findings:

  • B cell depletion therapy utilizing rituximab was administered to the CIDP-MGUS patient.
  • Rituximab treatment demonstrated a favorable impact on the patient's clinical symptoms.
  • Nerve conduction studies indicated improvement following rituximab therapy.

Implications:

  • B cell depletion with rituximab may represent a viable therapeutic option for refractory CIDP-MGUS.
  • Further research into rituximab's efficacy in CIDP-MGUS is warranted.
  • This case highlights a potential treatment strategy for this rare CIDP variant.