Chronic inflammatory demyelinating polyneuropathy (CIDP) [including IgG and IgA paraproteinaemic neuropathies]

Version: 2.1
Published: 09 July 2016
Condition for which Ig has an established therapeutic role.

This medical condition has either been superseded or has become inactive
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Specific Conditions
  • Chronic inflammatory demyelinating polyneuropathy
Indication for Ig Use
  • First-line treatment initiated when progression is rapid, or walking is compromised, or there is significant functional impairment.
Level of Evidence Clear evidence of benefit (Category 1)
Description and Diagnostic Criteria CIDP is an acquired sensorimotor polyneuropathy characterised by a progressive or relapsing/remitting course with evidence of demyelination on electrophysiological or pathological studies and response to immunomodulating therapies.

There is no specific diagnostic test, but characteristic clinical and laboratory findings help distinguish this disorder from other immune-mediated neuropathic syndromes. Serum protein electrophoresis with immunofixation may be indicated to search for monoclonal gammopathy and associated conditions.
Justification for Evidence Category The Biotext (2004) review found one Cochrane review of six randomised controlled trials (RCTs) with a total sample size of 170. The quality of the studies was low–moderate, found that intravenous immunoglobulin (IVIg) improved disability in the short term, and had comparable results to treatment with plasma exchange or prednisolone.

The Frommer and Madronio (2006) review found one low-quality RCT with a total sample size of 20, which demonstrated that more patients responded to immunoadsorption than IVIg, although the baseline disease duration was higher in the intravenous immunoglobulin (IVIg) group. Differences were not significant.
Diagnosis Requirements

A diagnosis must be made by any medical officer.

Where the diagnosis was not made by a Neurologist the diagnosis must be verified by a Neurologist.
Qualifying Criteria for Ig Therapy
  • Compromised walking or significant functional impairment as measured by activities of daily living (ADL) or other functional/disability scales.
 
Review Criteria for Assessing the Effectiveness of Ig Use
IVIg should be used for three to six months before determining whether the patient has responded. If there is no benefit after this treatment, IVIg therapy should be abandoned. Review by a Neurologist is required within six months and annually thereafter.

Documentation of clinical effectiveness is necessary for continuation of IVIg therapy.
 

On review of the initial authorisation period

Clinical effectiveness of Ig therapy can be demonstrated by:
 
  • Improvement in disability compared to the qualifying assessment, or stabilsation of disease after previous evidence of deterioration, as measured by activities of daily living (ADL) or other functional/disability scales.
     

On review of a continuing authorisation period

For stable patients on maintenance treatment, review by a Neurologist is required at least annually.

Clinical effectiveness of Ig therapy can be demonstrated by:
 
  • Improvement or stabilisation of disability compared to the previous review assessment as measured by activities of daily living (ADL) or other functional/disability scales.
Dose
  • Induction Dose (IVIg) - 2 g/kg in 2 to 5 divided doses.
  • Maintenance Dose (IVIg) - 0.4–1 g/kg, 2–6 weekly.
The amount per dose should be titrated to the individual's response. 

Aim for minimum dose to maintain optimal functional status.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Refer to the current product information sheet for further information.
Bibliography
Asia–Pacific IVIg Advisory Board 2004, ‘Bringing consensus to the use of IVIg in neurology. Expert consensus statements on the use of IVIg in neurology’, 1st edn, Asia–Pacific IVIg Advisory Board Inc., pp. 21–29.

Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy of intravenous immunoglobulin therapy and its risks, commissioned by the National Blood Authority on behalf of all Australian Governments, pp. 132–3. Available from: http://www.nba.gov.au/pubs/pdf/report-lit-rev.pdf

Frommer, M & Madronio, C 2006, The use of intravenous immunoglobulin in Australia. A report for the National Blood Authority, Part B, Sydney Health Projects Group, University of Sydney, Sydney, pp. 29–31.

Hughes, RAC, Bouche, P, Cornblath, DR, et al 2006, ‘European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society’, European Journal of Neurology, pp. 326–32.

van Schaik, IN, Winer, JB, de Haan, R, et al 2002, ‘Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy (Cochrane Review)’, in The Cochrane Library, Issue 2, John Wiley & Sons, Ltd, Chichester, UK.

Zinman, L, Sutton, HD, Ng, E, et al 2005, ‘A pilot study to compare the use of the Excorim staphylococcal protein immunoadsorption system and IVIG in chronic inflammatory demyelinating polyneuropathy’, Transfusion and Apheresis Science, vol. 33, no. 3, pp. 317–24.