Chronic Inflammatory Demyelinating Polyneuropathy Figure 1: Treatment Algorithm for Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Mild to moderate impairment or disability and
no contraindications for corticosteroids treatment and
no pure motor form of CIDP
Moderate to severe impairment or disability or
contraindication for corticosteroid treatment or
pure motor form of CIDP Very mild impairment or disability
One course of IVIg 2g/kg over 2 or 5 days
Insufficient improvement or deterioration
Repeat one course of IVIg 2g/kg over 2 or 5 days
Deterioration
Consider to repeat one course of IVIg Contraindication for corticosteroids?
No
Start dexamethasone 40mg for 4 days, repeated every month for six months
Alternative: prednisone 60mg/day during 6 weeks and taper dose over 32 weeks (dexamethasone acts faster and seems to have fewer long-term side effects)
Consider to add an immunosuppressive drug, see text Yes
Wait and see Monitor without treatment
Sufficient improvement, i.e. recovery to full or nearly full ability
Wait and see Monitor without treatment
No deterioration Remission
Repeat IVIg 1g/kg once every 3 weeks. Find lowest possible dose with longest possible intervals to maintain patient’s functional status, see text
Consider to add an immunosuppressive drug, see text
Patients who fail to respond to the initial treatment or have serious side effects should be switched to IVIg or corticosteroids (dexamethasone or prednisone). Plasma exchange can be useful in patients who do not respond to corticosteroids and IVIg. Consider to add an immunosuppressive drug, see text. Autologous stem cell transplantation can be tried in therapy resistant patients
CIDP = chronic inflammatory demyelinating polyradiculoneuropathy; IVIg = intravenous immunoglobulin. Tacrolimus
Tacrolimus is an immunosuppressant that inhibits both T-lymphocyte signal transduction and interleukin (IL)-2 transcription. One case report notes that tacrolimus had a positive effect in a patient with CIDP.100 Nephrotoxicity is the most important, although reversible, side effect. Many case reports and cases series have reported tacrolimus-related polyneuropathies often resembling CIDP, however.101–104
Interferon Beta
Three case reports showed improvement after treatment with different brands of interferon beta.105–107
of 24 CIDP patients, nine patients improved significantly;108,109
In two series, including a total however,
a randomised controlled trial including 10 treatment refractory CIDP patients did not show a significant effect on predetermined impairment and disability scales. Recently, these results were confirmed in a RCT with intramuscular interferon beta-1a, which, when added to IVIg, had no effect in CIDP.110
The most commonly seen adverse events are flu-like symptoms, mild leucocytopenia and alteration of liver function. Two case reports of patients with multiple sclerosis developing CIDP after interferon beta-1b treatment have been published.111,112
These adverse events, 48
together with its doubtful efficacy and high costs, must lead to the conclusion that there is no place for interferon beta treatment in CIDP.
Interferon Alpha
Nine of 14 patients with CIDP treated with subcutaneous interferon alpha-2a three times a week for six weeks had a favourable response, although three patients later had a relapse.113 reports confirming these beneficial results.114–116
There are a few case Adverse events are
similar to those seen with interferon beta, but costs are lower. Development of CIDP upon treatment with interferon alpha has also been reported, however.117,118
Haematopoietic Autologous Stem Cell Transplantation Autologous stem cell transplantation was used successfully in CIDP for the first time in 2001.119
Since this first case report, successful
treatment with autologous stem cell transplantation has been reported in several patients with CIDP refractory to other treatments.83,120–122
three patients with CIDP improved.123
In an open label non-randomised study, two of One patient relapsed five years
after transplantation. This patient was no longer refractory to treatment and responded well to normal doses of IVIg.124
One case report describes a patient who developed CIDP after autologous stem EUROPEAN NEUROLOGICAL REVIEW
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