Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

no significant impact on function and quality of life

1st line – observation

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ACUTE
|
no significant impact on function and quality of life
1st line – 

observation

Patients with mild disease (weakness that does not interfere with daily activities) may be monitored for deterioration without treatment.[131]​​​

significant impact on function and quality of life

1st line – initial monotherapy: IVIG, corticosteroid, or plasma exchange

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ACUTE
|
significant impact on function and quality of life
1st line – 

initial monotherapy: IVIG, corticosteroid, or plasma exchange

Approximately 75% to 85% of patients will respond to monotherapy with intravenous immunoglobulin (IVIG), corticosteroids, or plasma exchange.[1]​​​​[58][60][132][133][134][135]

Corticosteroids are effective for treating CIDP.[1][60][134]​ High-dose pulsed methylprednisolone or dexamethasone are often tried first as they are easy to administer, have few adverse effects, have rapid onset of benefit, and are inexpensive.[1][134][136]​​​​​ Efficacy and improvement in disability after treatment with pulsed high-dose dexamethasone or typical daily prednisolone regimens are equivalent; however, daily oral prednisolone may cause many more adverse effects, and may take longer to show benefit.[1][134]​​​[135][136]

IVIG is an effective option, with ease of use, rapid onset of benefit, and low incidence of adverse effects.[1][59][131][132][135]​​[137]​​​ There is no evidence for a difference in treatment efficacy between IVIG preparations.[1][138]​​ If efficacious, IVIG can be repeated from every 2 to 6 weeks.[1] To optimise therapy, the dose should be reduced before the frequency of administration is lowered, to achieve maximal benefits with minimal amounts of IVIG. However, care must be taken to avoid deterioration before the next dose.[1]​​[131][132]

Plasma exchange is effective and relatively safe, resulting in significant short-term improvement in disability, clinical impairment, and motor nerve conduction velocity in patients with CIDP.[1][133]​ However, some patients who show improvement subsequently deteriorate.[133] Plasma exchange requires good vascular access and specialised equipment, which can make it less convenient than other treatments, especially in paediatric patients.[1][133][139]​ Initial regimen is generally 5 exchanges over 2 weeks, with further dosing based on response.

Randomised controlled trials comparing efficacy between corticosteroids, IVIG, and plasma exchange do not show a significant benefit of any one over the others.[1][2][135]​ The final decision on which treatment to use is determined by availability, risks, contraindications, and disease severity; however, because corticosteroids and IVIG are easier to administer and are generally better tolerated, they are usually recommended ahead of plasma exchange.[1] If significant adverse effects occur with an initial therapy, an alternative should be substituted.

Corticosteroid therapy is not recommended as first-line treatment for patients with motor CIDP, due to evidence of deterioration after such therapy.[1]​​[68][69][135]

Primary options

methylprednisolone: 500 mg intravenously once daily for 4 days every month for 6 months

More

OR

dexamethasone: 40 mg orally once daily for 4 days every month for 6 months

OR

prednisolone: 1-2 mg/kg/day orally until clinical response (usually 4-12 weeks), followed by slow taper over months, maximum 60 mg/day

OR

normal immunoglobulin human: 2 g/kg intravenously initially given in divided doses over 2-5 days

More

Consider – pharmacotherapy for neuropathic pain

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ACUTE
|
significant impact on function and quality of life
Consider – 

pharmacotherapy for neuropathic pain

Additional treatment recommended for SOME patients in selected patient group

All patients with neuropathic pain should be offered symptomatic therapy.

Based on current guidelines, pregabalin, gabapentin, tricyclic antidepressants (e.g., amitriptyline), and serotonin-noradrenaline reuptake inhibitors (venlafaxine or duloxetine) are commonly used as first-line agents.[1]​​[157][158]

Tramadol and other opioid analgesics (e.g., oxycodone) are recommended as second- and third-line agents, respectively, for neuropathic pain.[157] Guidance from the US Centers for Disease Control and Prevention notes that evidence for efficacy of opioid therapy for neuropathic pain is limited, and that opioids should only be considered once other options have been tried, and if the expected benefits are anticipated to outweigh risks to the patient.[159]

With all of these medicines, a slow upward titration of dose may help to avoid adverse effects, especially those related to sedation.

Primary options

gabapentin: 300-1200 mg orally three times daily

OR

pregabalin: 50-100 mg orally three times daily

OR

amitriptyline: 10-150 mg orally once daily at bedtime

OR

venlafaxine: 75-225 mg orally (extended-release) once daily

OR

duloxetine: 30-60 mg orally once daily

Secondary options

tramadol: 50-100 mg orally (immediate-release) every 4-6 hours when required

Tertiary options

oxycodone: 5-15 mg orally (immediate-release) every 4-6 hours when required

Consider – allied health referral

Back
ACUTE
|
significant impact on function and quality of life
Consider – 

allied health referral

Additional treatment recommended for SOME patients in selected patient group

Depending on the severity of the weakness, physiotherapy, occupational therapy, and orthotic evaluation may be needed.

Referral to one of more of a physical medicine and rehabilitation physician, a psychiatrist or psychologist, a pain management consultant, and a podiatrist should be considered on an individual basis.[1]

A home exercise programme under the supervision of a physiotherapist may be of benefit.[162]

Reducing sedative use, better sleep hygiene, and treating depression may help with CIDP-related fatigue.[160][161]

partial or no response to initial monotherapy

1st line – combination therapy with 2 initial agents

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ACUTE
|
partial or no response to initial monotherapy
|
partial response
1st line – 

combination therapy with 2 initial agents

If there is a partial but insufficient objective response to the initial agent (i.e., daily activities are still affected by distal weakness and paraesthesias), this agent should be continued with a second initial agent added to the regimen.[1]

Choice of combination therapy is based on availability, risks, and the severity of disease. See above for more information about treatment options (i.e., initial monotherapy: IVIG, corticosteroids, or plasma exchange).

Consider – pharmacotherapy for neuropathic pain

Back
ACUTE
|
partial or no response to initial monotherapy
|
partial response
Consider – 

pharmacotherapy for neuropathic pain

Additional treatment recommended for SOME patients in selected patient group

All patients with neuropathic pain should be offered symptomatic therapy.

Based on current guidelines, pregabalin, gabapentin, tricyclic antidepressants (e.g., amitriptyline), and serotonin-noradrenaline reuptake inhibitors (venlafaxine or duloxetine) are commonly used as first-line agents.[1]​​[157][158]

Tramadol and other opioid analgesics (e.g., oxycodone) are recommended as second- and third-line agents, respectively, for neuropathic pain.[157] Guidance from the US Centers for Disease Control and Prevention notes that evidence for efficacy of opioid therapy for neuropathic pain is limited, and that opioids should only be considered once other options have been tried, and if the expected benefits are anticipated to outweigh risks to the patient.[159]

With all of these medicines, a slow upward titration of dose may help to avoid adverse effects, especially those related to sedation.

Primary options

gabapentin: 300-1200 mg orally three times daily

OR

pregabalin: 50-100 mg orally three times daily

OR

amitriptyline: 10-150 mg orally once daily at bedtime

OR

venlafaxine: 75-225 mg orally (extended-release) once daily

OR

duloxetine: 30-60 mg orally once daily

Secondary options

tramadol: 50-100 mg orally (immediate-release) every 4-6 hours when required

Tertiary options

oxycodone: 5-15 mg orally (immediate-release) every 4-6 hours when required

Consider – allied health referral

Back
ACUTE
|
partial or no response to initial monotherapy
|
partial response
Consider – 

allied health referral

Additional treatment recommended for SOME patients in selected patient group

Depending on the severity of the weakness, physiotherapy, occupational therapy, and orthotic evaluation may be needed.

Referral to one or more of a physical medicine and rehabilitation physician, a psychiatrist or psychologist, a pain management consultant, and a podiatrist should be considered on an individual basis.[1]

A home exercise programme under the supervision of a physiotherapist may be of benefit.[162]

Reducing sedative use, better sleep hygiene, and treating depression may help with CIDP-related fatigue.[160][161]

1st line – alternative initial agent

Back
ACUTE
|
partial or no response to initial monotherapy
|
no response
1st line – 

alternative initial agent

It may take up to 3 months to determine treatment effectiveness. Lack of at least a partial response to one or two initial agents should lead to reconsideration of the diagnosis.[2]

If there is no objective response to monotherapy with an initial agent within a few weeks to months, an alternative initial agent (i.e., corticosteroids, IVIG, or plasma exchange) should be tried. See above for more information about treatment options (i.e., initial monotherapy: IVIG, corticosteroids, or plasma exchange).

Consider – pharmacotherapy for neuropathic pain

Back
ACUTE
|
partial or no response to initial monotherapy
|
no response
Consider – 

pharmacotherapy for neuropathic pain

Additional treatment recommended for SOME patients in selected patient group

All patients with neuropathic pain should be offered symptomatic therapy.

Based on current guidelines, pregabalin, gabapentin, tricyclic antidepressants (e.g., amitriptyline), and serotonin-noradrenaline reuptake inhibitors (venlafaxine or duloxetine) are commonly used as first-line agents.[1]​​[157][158]

Tramadol and other opioid analgesics (e.g., oxycodone) are recommended as second- and third-line agents, respectively, for neuropathic pain.[157] Guidance from the US Centers for Disease Control and Prevention notes that evidence for efficacy of opioid therapy for neuropathic pain is limited, and that opioids should only be considered once other options have been tried, and if the expected benefits are anticipated to outweigh risks to the patient.[159]

With all of these medicines a slow upward titration of dose may help to avoid adverse effects, especially those related to sedation.

Primary options

gabapentin: 300-1200 mg orally three times daily

OR

pregabalin: 50-100 mg orally three times daily

OR

amitriptyline: 10-150 mg orally once daily at bedtime

OR

venlafaxine: 75-225 mg orally (extended-release) once daily

OR

duloxetine: 30-60 mg orally once daily

Secondary options

tramadol: 50-100 mg orally (immediate-release) every 4-6 hours when required

Tertiary options

oxycodone: 5-15 mg orally (immediate-release) every 4-6 hours when required

Consider – allied health referral

Back
ACUTE
|
partial or no response to initial monotherapy
|
no response
Consider – 

allied health referral

Additional treatment recommended for SOME patients in selected patient group

Depending on the severity of the weakness, physiotherapy, occupational therapy, and orthotic evaluation may be needed.

Referral to one of more of a physical medicine and rehabilitation physician, a psychiatrist or psychologist, a pain management consultant, and a podiatrist should be considered on an individual basis.[1]

A home exercise programme under the supervision of a physiotherapist may be of benefit.[162]

Reducing sedative use, better sleep hygiene, and treating depression may help with CIDP-related fatigue.[160][161]

refractory to combination therapy with 2 initial agents

1st line – continue initial agent that produced partial response

Back
ACUTE
|
refractory to combination therapy with 2 initial agents
1st line – 

continue initial agent that produced partial response

For patients whose condition does not improve sufficiently in response to a combination of two initial agents (i.e. two of corticosteroids, IVIG, and plasma exchange), re-evaluation of the diagnosis and referral to a specialist centre is appropriate.​[16][42]

The initial agent to which a patient has shown a partial response should be continued. See above for more information about treatment options (i.e., initial monotherapy: IVIG, corticosteroids, or plasma exchange).

Plus – addition of alternative immunosuppressant

Back
ACUTE
|
refractory to combination therapy with 2 initial agents
Plus – 

addition of alternative immunosuppressant

Treatment recommended for ALL patients in selected patient group

Once the diagnosis of CIDP is confirmed, an alternative immunosuppressant should be added. Although there is limited evidence, ciclosporin, rituximab, cyclophosphamide, azathioprine, or mycophenolate may be considered as an alternative agent after failure of initial treatments, or as add-on medication.[1][141][142]​​ Any combination of initial and alternative agents can be used, and decisions should be based on severity of disease and adverse-effect profile.[1]

Guidelines recommend against using interferon beta-1a, methotrexate, fingolimod, alemtuzumab, bortezomib, etanercept, fampridine, fludarabine, immunoadsorption, interferon alfa, abatacept, natalizumab, and tacrolimus.[1]

In one retrospective analysis, approximately 25% of patients refractory to standard therapy showed a response to an alternative immunosuppressant; the authors noted that adverse effects should be monitored closely.[142]

Ciclosporin is well tolerated, drug levels can be monitored to guide treatment, and it usually produces benefit within 3-6 months.[1][141] Combining ciclosporin with plasma exchange can make it difficult to achieve therapeutic levels of ciclosporin.

Cyclophosphamide (often in combination with a corticosteroid) may be used to treat refractory disease.[1] In one meta-analysis, cyclophosphamide yielded a response rate of 68% in patients with refractory CIDP; however, in practice, cyclophosphamide should be used with caution due to its toxicity, and patients must be monitored for adverse effects.[143]

Rituximab may be considered in refractory CIDP or CIDP associated with other autoimmune diseases or haematological diseases (e.g. monoclonal gammopathy).​[62][141]​​[144][145]​​​​ Rituximab may also be considered with nodal/paranodal antibodies (e.g. IgG4 anti-contactin-1 or anti-neurofascin-155 antibodies) after failure of corticosteroids or IVIG.[64][104][146][147]​​​​ It is recommended for children instead of cyclophosphamide because of a better adverse-effect profile.[1]

Azathioprine is usually well tolerated.[1][141]​​​​​[148]​ However, onset of action may take 6-18 months, so it is not recommended for initial treatment of relapse or active refractory cases unless combined with other agents.

Mycophenolate is used similarly to azathioprine as an additional agent when rapid improvement is not needed.[1] Some, but not all, studies have shown benefit.[149][150]

Primary options

ciclosporin: consult specialist for guidance on dose

OR

rituximab: consult specialist for guidance on dose

OR

cyclophosphamide: consult specialist for guidance on dose

Secondary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

Consider – pharmacotherapy for neuropathic pain

Back
ACUTE
|
refractory to combination therapy with 2 initial agents
Consider – 

pharmacotherapy for neuropathic pain

Additional treatment recommended for SOME patients in selected patient group

All patients with neuropathic pain should be offered symptomatic therapy.

Based on current guidelines, pregabalin, gabapentin, tricyclic antidepressants (e.g., amitriptyline), and serotonin-noradrenaline reuptake inhibitors (venlafaxine or duloxetine) are commonly used as first-line agents.​[1][157][158]

Tramadol and other opioid analgesics (e.g., oxycodone) are recommended as second- and third-line agents, respectively, for neuropathic pain.[157] Guidance from the US Centers for Disease Control and Prevention notes that evidence for efficacy of opioid therapy for neuropathic pain is limited, and that opioids should only be considered once other options have been tried, and if the expected benefits are anticipated to outweigh risks to the patient.[159]

With all of these medicines, a slow upward titration of dose may help to avoid adverse effects, especially those related to sedation.

Primary options

gabapentin: 300-1200 mg orally three times daily

OR

pregabalin: 50-100 mg orally three times daily

OR

amitriptyline: 10-150 mg orally once daily at bedtime

OR

venlafaxine: 75-225 mg orally (extended-release) once daily

OR

duloxetine: 30-60 mg orally once daily

Secondary options

tramadol: 50-100 mg orally (immediate-release) every 4-6 hours when required

Tertiary options

oxycodone: 5-15 mg orally (immediate-release) every 4-6 hours when required

Consider – allied health referral

Back
ACUTE
|
refractory to combination therapy with 2 initial agents
Consider – 

allied health referral

Additional treatment recommended for SOME patients in selected patient group

Depending on the severity of the weakness, physiotherapy, occupational therapy, and orthotic evaluation may be needed.

Referral to one or more of a physical medicine and rehabilitation physician, a psychiatrist or psychologist, a pain management consultant, and a podiatrist should be considered on an individual basis.[1]

A home exercise programme under the supervision of a physiotherapist may be of benefit.[162]

Reducing sedative use, better sleep hygiene, and treating depression may help with CIDP-related fatigue.[160][161]

ONGOING

response to treatment

1st line – maintenance therapy

Back
ONGOING
|
response to treatment
1st line – 

maintenance therapy

Maintenance therapy may require long-term use of an initial agent, an alternative agent, or a combination of two or more initial or alternative agents, depending on which treatments have produced a response in the acute phase of treatment. IVIG and corticosteroids are the most common agents used. Plasma exchange is generally not recommended long term for patients in whom immunoglobulin and corticosteroids are ineffective, because of the need for vascular access.[1]

With a good response to an initial agent, the patient usually has up to 1-2 years of therapy (depending on agent used) with a slow taper. If there is a disease exacerbation during taper, the dose should be increased to the initial dose that resulted in a good response.[1] Some patients may require 5-15 years of treatment, but eventual withdrawal should be considered as approximately one-third of patients will go into remission and not require subsequent therapy. Thus, the need for continued maintenance therapy should be re-assessed every 6-12 months.[1]

IVIG as maintenance treatment is associated with few adverse effects. The optimal dose and interval for IVIG maintenance treatment are not known. Once the patient is stable, treatment can be tapered based on clinical experience, by either reducing the dose or increasing treatment interval. The adjustment should be done every 6-12 months in the first 2-3 years of treatment.[1]​ In patients with a complete or near-complete response (distal weakness may not fully improve), IVIG should be tapered off and a trial on no immunosuppressants should be instituted.

Subcutaneous immunoglobulin (SCIG) is approved as CIDP maintenance therapy in patients on a stable dose of IVIG. SCIG is typically given once weekly, but can be given over 2-3 days for larger doses. There is insufficient evidence that a higher dose of SCIG is superior to a lower dose, but the relapse rate was lower in the higher-dose group.[1][151][152]​​ When switching from IVIG to SCIG, it is advised to use the same mean dose per week.[1] SCIG may be beneficial for patients with adverse effects or treatment-related fluctuations from IVIG that are not resolved by premedication or dose adjustment.[153]​ SCIG can be administered at home by the patient, and eliminates problems associated with venous access. Some patients may, however, find it difficult to self-administer SCIG due to weakness; others may experience local infusion site reactions.

Long-term corticosteroid treatment may induce significant adverse effects (e.g., osteoporosis, gastric ulceration, diabetes mellitus, cataracts, avascular necrosis of long bones, arterial hypertension). However, high-dose pulsed corticosteroids are associated with fewer adverse effects than daily oral dosing.[1] A corticosteroid taper may involve lowering the dose, reducing the frequency, or both; refer to local guidance on tapers.

Ciclosporin, azathioprine, and mycophenolate can all be used as alternative agents to decrease the dose or frequency of corticosteroids or immunoglobulin, or when patients experience adverse effects from those therapies, although evidence of effectiveness is of low certainty.[1]

Ciclosporin usually shows benefit within 3-6 months, while azathioprine and mycophenolate may take up to 6-18 months. They may all increase the risk of malignancy when used for >10 years. Other alternative agents should only be considered after these drugs have been shown to be ineffective.

Due to lack of evidence of efficacy and/or adverse safety profiles, guidelines recommend against using the following agents: methotrexate, tacrolimus, interferon beta-1a, interferon alfa, fingolimod, alemtuzumab, bortezomib, natalizumab, etanercept, abatacept, fampridine, fludarabine, and immunoadsorption.[1]

Consider – pharmacotherapy for neuropathic pain

Back
ONGOING
|
response to treatment
Consider – 

pharmacotherapy for neuropathic pain

Additional treatment recommended for SOME patients in selected patient group

All patients with neuropathic pain should be offered symptomatic therapy.

Based on current guidelines, pregabalin, gabapentin, tricyclic antidepressants (e.g., amitriptyline), and serotonin-noradrenaline reuptake inhibitors (venlafaxine or duloxetine) are commonly used as first-line agents.[1]​​[157][158]

Tramadol and other opioid analgesics (e.g., oxycodone) are recommended as second- and third-line agents, respectively, for neuropathic pain.[157] Guidance from the US Centers for Disease Control and Prevention notes that evidence for efficacy of opioid therapy for neuropathic pain is limited, and that opioids should only be considered once other options have been tried, and if the expected benefits are anticipated to outweigh risks to the patient.[159]

With all of these medicines, a slow upward titration of dose may help to avoid adverse effects, especially those related to sedation.

Primary options

gabapentin: 300-1200 mg orally three times daily

OR

pregabalin: 50-100 mg orally three times daily

OR

amitriptyline: 10-150 mg orally once daily at bedtime

OR

venlafaxine: 75-225 mg orally (extended-release) once daily

OR

duloxetine: 30-60 mg orally once daily

Secondary options

tramadol: 50-100 mg orally (immediate-release) every 4-6 hours when required

Tertiary options

oxycodone: 5-15 mg orally (immediate-release) every 4-6 hours when required

Consider – allied health referral

Back
ONGOING
|
response to treatment
Consider – 

allied health referral

Additional treatment recommended for SOME patients in selected patient group

Depending on the severity of the weakness, physiotherapy, occupational therapy, and orthotic evaluation may be needed.

Referral to one of more of a physical medicine and rehabilitation physician, a psychiatrist or psychologist, a pain management consultant, and a podiatrist should be considered on an individual basis.[1]

A home exercise programme under the supervision of a physiotherapist may be of benefit.[162]

Reducing sedative use, better sleep hygiene, and treating depression may help with CIDP-related fatigue.[160][161]

no response to treatment

1st line – supportive care

Back
ONGOING
|
no response to treatment
1st line – 

supportive care

Absence of response to any treatment is highly unlikely. If this happens, the diagnosis should be reviewed. Nearly all patients respond, at least partially, to some kind of pharmacological therapy.

Supportive care is the standard treatment for patients with an incomplete response.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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