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Immunoglobulins in chronic inflammatory demyelinating polyneuropathy
1Department of Neurology, Università Vita-Salute and Scientific Institute San Raffaele, Via Olgettina 60, I-20132, Milan, Italy.
Summary
Intravenous immunoglobulins (IVIg) effectively treat chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), with most patients needing ongoing treatment. IVIg shows some efficacy in paraproteinemic demyelinating neuropathy (PDN), but less consistently.
Area of Science:
- Neurology
- Immunology
- Clinical Therapeutics
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Paraproteinemic demyelinating neuropathy (PDN) is a CIDP subtype associated with monoclonal gammopathy.
- Intravenous immunoglobulins (IVIg) are a therapeutic option for various immune-mediated conditions.
Purpose of the Study:
- To review the efficacy and tolerability of IVIg in CIDP.
- To evaluate IVIg effectiveness specifically in PDN.
- To assess the safety profile of IVIg treatment in these neuropathies.
Main Methods:
- Review of Class I clinical trials for IVIg in CIDP.
- Analysis of evidence for IVIg efficacy in PDN.
- Assessment of reported safety data for IVIg.
Main Results:
- Class I trials confirm IVIg effectiveness in CIDP.
- Approximately two-thirds of CIDP patients respond to initial IVIg treatment.
- Less than 50% of PDN patients respond to IVIg, with long-term persistence unclear.
- IVIg generally has a good safety profile, but serious adverse effects are possible.
Conclusions:
- IVIg is an effective treatment for CIDP, often requiring maintenance therapy.
- IVIg shows potential for PDN, but response rates are lower and less understood.
- Careful consideration of potential serious adverse effects is crucial before initiating IVIg therapy.