To All Those With Chronic Inflammatory Demyelinating Polyneuropathy

Recently, one of the physicians on the GBS/CIDP Foundation Medical Advisory Board saw a man who carried the diagnosis of CIDP for 2 years. During that time, he received IVIg monthly for 18 months then plasmapheresis monthly for five months without any clinical improvement. He went to a GBS/CIDP Foundation Center of Excellence where it was determined that he did not have CIDP, as the diagnosis was made on incorrectly interpreted nerve conduction studies in the wrong clinical context. The patient calculated that the cost of his treatments was over $400,000. Unfortunately, this is not uncommon.

Members of the Medical Advisory Board have been giving direct-to-patient talks on CIDP to Foundation patient support groups around the country. The main message is that the diagnosis and treatment of CIDP can and must be improved.

We want those with CIDP to:

  • Get the correct diagnosis,
  • Get the right treatment, and then
  • Get off treatment if treatment is no longer necessary.

It is important to know what CIDP is. Typical CIDP is a spectrum of polyneuropathies characterized by:

  • Development and progression for at least 2 months,
  • Sensory and motor symptoms,
  • Distal sensory loss, distal and proximal limb weakness, and areflexia on neurologic examination,
  • Demyelination on nerve conduction studies,
  • Elevated CSF protein without increase in cell count,
  • Normal routine blood studies including protein studies,
  • Nerve biopsy, if done, demonstrating T cell infiltration and macrophage-associated demyelination, and
  • Clear response to treatment(s) that have been demonstrated to be effective in CIDP.

There are 3 proven first line treatments for CIDP: IVIg, plasmapheresis and corticosteroids. The majority of people with true CIDP improve with one of these treatments. A clear response to any of these treatments is almost always seen within 2-3 months; no response after several months suggests rethinking the diagnosis and not continuing with that particular therapy.

Depending on the response to the first line treatments, other treatments may not be needed, but if other drugs are needed, a large number have been used successfully. Once on a successful treatment, it should be discontinued slowly to determine if it is still needed, because clinical trials have shown that 40% or more of those on treatments for CIDP do not require long-term therapy.

We recommend that you obtain another opinion for your CIDP from a Center of Excellence if:

  • You do not have the typical features of CIDP described above but are receiving treatment(s) for CIDP.
  • CIDP was diagnosed mainly based on nerve conduction studies.
  • CIDP was diagnosed mainly based on elevated spinal fluid protein.
  • You are not clearly improving on your current treatment(s).
  • You have been on treatments for a long time and are stable.

We hope these recommendations improve the care and treatment of those with CIDP. Questions can be directed to the Foundation.

David R. Cornblath, MD
Member, Medical Advisory Board
Member, Board of Directors

Kenneth C. Gorson, MD
Member, Medical Advisory Board

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