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

Version: 3.3
Published: 30 November 2023
Condition for which Ig has an established therapeutic role.

This indication should be used to cover first line (prior to trial off) treatment of CIDP patients. For patients who have trialled off Ig therapy and subsequently relapse within six months, please use the indication Relapse of chronic inflammatory demyelinating polyneuropathy (CIDP) patients within six months of commencement of trial off Ig therapy
 
In adults or children 10 years or older

• Significant disability or compromised walking in an adult or child 10 years or older as objectively measured by an Overall Neuropathy Limitations Scale (ONLS) score of at least two points and the Medical Research Council (MRC) sum score

In children less than 10 years

• Significant disability or compromised walking in a child less than 10 years as measured by the Six Minute Walk Test (6MWT) and/or a Modified Rankin Scale (MRS) score of at least two points

Review by a neurologist is required after four months of Ig therapy to determine whether the patient has responded. If there is no benefit after this period of treatment, IVIg therapy should be abandoned.
 
Where treatment is continued, a review by a neurologist or general physician is required each 12 months.
 
A trial of cessation should be considered each 12 months in patients in remission on maintenance therapy.
 
Once a patient has relapsed in the first six months of a trial off therapy, a further trial might be considered after at least two years.
 
Documentation of clinical efficacy is necessary for continuation of IVIg therapy.
 

This indication should be used for patients who have previously trialled off Ig therapy and subsequently relapsed within six months. For patients requiring first line therapy (i.e. prior to trial off please use the indication: Treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) for patients in whom walking is compromised or there is significant disability
 

• In a previously stable adult or child (10 years or older), deterioration in disability as measured by an increase of at least one point in the Adjusted Overall Neuropathy Limitations Scale (ONLS) or a reduction in the Medical Research Council (MRC) sum score of at least three points, when compared to the review score before stopping previous treatment
• OR
• In a previously stable child (less than 10 years), deterioration in disability as measured by a reduction in the Six Minute Walk Test (6MWT) or an increase of at least one point in the Modified Rankin Scale (MRS) compared to the review score before stopping previous treatment

• Relapse has occurred within six months of the last immunoglobulin dose

IVIg should be used for a maximum period of four months (induction plus three maintenance cycles) 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 four months and annually thereafter (neurologist or general physician).
 
Documentation of clinical efficacy is necessary for continuation of IVIg therapy.
 
Once a patient has relapsed when trialled off Ig treatment, a second line immunomodulatory agent should be strongly considered as additional therapy.

Review by a neurologist is required after four months of Ig therapy to determine whether the patient has responded. If there is no benefit after this period of treatment, IVIg therapy should be abandoned.
 
Where treatment is continued, a review by a neurologist or general physician is required each 12 months.
 
A trial of cessation should be considered each 12 months in patients in remission on maintenance therapy.
 
Once a patient has relapsed in the first six months of a trial off therapy, a further trial might be considered after at least two years.
 
Documentation of clinical efficacy is necessary for continuation of IVIg therapy.
 
Clinical effectiveness of Ig therapy may be demonstrated by:
 

On review of the initial authorisation period

For adults or children 10 years or older

• Improvement in disability as measured by a reduction in the Adjusted Overall Neuropathy Limitations Scale (ONLS) by at least one point; or by an increase in the Medical Research Council (MRC) sum score by at least three points, as compared to the qualifying assessment

For children less than 10 years

• Improvement in disability as measured by the Six Minute Walk Test (6MWT) and/or the Modified Rankin Scale (MRS) as compared to the qualifying assessment

On review of a continuing authorisation period

• In an adult or child 10 years or older, stabilisation or continued improvement in disease as measured by the adjusted Overall Neuropathy Limitations Scale (ONLS) or the Medical Research Council (MRC) sum score compared to the previous review score
   
• OR
• Adult patient with severe disease continues to report post infusion improvement that is better or comparable to the level reported at the previous review, with end-of-cycle deterioration and additional immunosuppressant agents have been commenced.
• OR
• In a child under 10, stabilisation or continued improvement in disease after previous evidence of deterioration in the Six Minute Walk Test (6MWT) or the Modified Rankin Scale (MRS) compared to the previous review scores

• A trial of weaning towards cessation of Ig therapy is planned for clinically stable patients to identify those in remission, or a valid reason provided as to why a trial is not planned or is contraindicated at this time is provided

A trial of Ig weaning should be considered annually in stable patients on maintenance therapy to identify patients who are in remission.

IVIg should be used for a maximum period of four months (induction plus three maintenance cycles) 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 four months and annually thereafter (neurologist or general physician).
 
Documentation of clinical efficacy is necessary for continuation of IVIg therapy.
 
Once a patient has relapsed when trialled off Ig treatment, a second line immunomodulatory agent should be strongly considered as additional therapy.
 
Clinical effectiveness of Ig therapy may be demonstrated by:
 

On review of the initial authorisation period

• In an adult or child (10 years or older), improvement in disability as measured by a decrease of at least one point in the Adjusted Overall Neuropathy Limitations Scale (ONLS) score or an increase of at least three points in the Medical Research Council (MRC) sum score
• OR
• In a child (less than 10 years), improvement in disability as measured by improvement in the Six Minute Walk Test (6MWT) or a reduction of at least one point in the Modified Rankin Scale (MRS) score

• A trial of weaning towards cessation of Ig therapy is planned for patients who are clinically stable to identify those in remission or a valid reason provided as to why a trial is not being planned or is contraindicated at this time

On review of a continuing authorisation period

• In an adult or child (10 years or older), stabilisation or continued improvement in disease  as measured by the Overall Neuropathy Limitations Scale (ONLS) score, or the MRC score, as compared to the previous assessment
• OR
• Adult patient with severe disease continues to report post infusion improvement that is better or comparable to the level reported at the previous assessment, with end-of-cycle deterioration and additional immunosuppressant agents have been commenced
• OR
• In a child (less than 10 years), stabilisation or continued improvement in disease after previous evidence of deterioration as measured by the Six Minute Walk Test (6MWT) or the Modified Rankin Scale (MRS) score

• A trial of Ig weaning towards cessation of Ig therapy is planned for patients who are clinically stable to identify those in remission or a valid reason provided as to why a trial is not being planned or is contraindicated at this time

Once a patient has relapsed in the first six months of a trial off therapy, a further trial might be considered after at least two years.

• Loading Dose (IVIg) - One loading dose of 2 g/kg in 2 to 5 divided doses in the first month of therapy (in addition to the maintenance dose) is permitted.
  This dose is only available as intravenous immunoglobulin
• Maintenance Dose (IVIg) - 0.4–1g/kg, is permitted every 2 to 6 weeks. The amount per dose should be titrated to the individual’s response, and may be reduced while weaning. A maximum dose of 2g/kg may be given in any 4 week period. This might be by smaller doses more frequently than fortnightly.
  Subcutaneous administration of immunoglobulin can be considered as an alternative to intravenous immunoglobulin following stabilisation with intravenous immunoglobulin.
• Supplementary Dose (IVIg) - One additional dose of up to 2 g/kg, administered in divided doses where appropriate, may be given where there is acute deterioration/relapse in the context of a severe infection, surgery or other acute illness. Deterioration of a patient on Ig therapy may be an indication for alternative or additional immunosuppressant agents.
  Access to an additional dose should be a rare occurrence.
  
  This dose is also available as subcutaneous immunoglobulin.
• Maintenance Dose (SCIg) - 0.2–0.5g/kg, is permitted every 1 week. The amount per dose should be titrated to the individual’s response, and may be reduced while weaning. A maximum dose of 2g/kg may be given in any 4 week period. This might be by smaller doses more frequently than weekly.
  This dose is also available as intravenous immunoglobulin.
• Supplementary Dose (SCIg) - One additional dose of up to 2 g/kg, administered in divided doses where appropriate, may be given where there is acute deterioration/relapse in the context of a severe infection, surgery or other acute illness. Deterioration of a patient on Ig therapy may be an indication for alternative or additional immunosuppressant agents.
  Access to an additional dose should be a rare occurrence.
  
  This dose is also available as intravenous immunoglobulin.

• Loading Dose (IVIg) - One loading dose of 2 g/kg in 2 to 5 divided doses in the first month of therapy (in addition to the maintenance dose) is permitted.
  This dose is only available as intravenous immunoglobulin
• Maintenance Dose (IVIg) - 0.4–1g/kg, is permitted every 2 to 6 weeks. The amount per dose should be titrated to the individual’s response, and may be reduced while weaning. A maximum dose of 2g/kg may be given in any 4 week period. This might be by smaller doses more frequently than fortnightly.
  Subcutaneous administration of immunoglobulin can be considered as an alternative to intravenous immunoglobulin following stabilisation with intravenous immunoglobulin.
• Supplementary Dose (IVIg) - One additional dose of up to 2 g/kg, administered in divided doses where appropriate, may be given where there is acute deterioration/relapse in the context of a severe infection, surgery or other acute illness. Deterioration of a patient on Ig therapy may be an indication for alternative or additional immunosuppressant agents.
  Access to an additional dose should be a rare occurrence.
  
  This dose is also available as subcutaneous immunoglobulin.
• Maintenance Dose (SCIg) - 0.2–0.5g/kg, is permitted every 1 week. The amount per dose should be titrated to the individual’s response, and may be reduced while weaning. A maximum dose of 2g/kg may be given in any 4 week period. This might be by smaller doses more frequently than weekly.
  This dose is also available as intravenous immunoglobulin.
• Supplementary Dose (SCIg) - One additional dose of up to 2 g/kg, administered in divided doses where appropriate, may be given where there is acute deterioration/relapse in the context of a severe infection, surgery or other acute illness. Deterioration of a patient on Ig therapy may be an indication for alternative or additional immunosuppressant agents.
  Access to an additional dose should be a rare occurrence.
  
  This dose is also available as intravenous immunoglobulin.