Chronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy: Treatment and prognosis - UpToDate 25/04/2019, 16+40
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Chronic inflammatory demyelinating polyneuropathy:

Treatment and prognosis
Author: Richard A Lewis, MD
Section Editor: Jeremy M Shefner, MD, PhD
Deputy Editor: John F Dashe, MD, PhD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Mar 2019. | This topic last updated: Dec 06, 2017.
INTRODUCTION
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an entity that describes a

group of related neuropathies, all having chronicity, demyelination, inflammation, and immune-
mediation in common.
In the classic form of CIDP, motor involvement is greater than sensory, and neurologic deficits
are fairly symmetric. Weakness is present in both proximal and distal muscles. Most patients
have globally diminished or absent reflexes. Cranial nerve and bulbar involvement occur in a
minority. The clinical course of CIDP is slowly progressive in a majority of patients, but a
relapsing-remitting course is noted in at least one-third.
Of major importance, CIDP generally responds to immunomodulatory treatment with

glucocorticoids, intravenous immune globulin, or plasma exchange.
The treatment and prognosis of CIDP will be reviewed here. Other aspects of CIDP are
discussed separately. (See "Chronic inflammatory demyelinating polyneuropathy: Etiology,
clinical features, and diagnosis".)
APPROACH TO TREATMENT
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There are some patients with CIDP who have such mild disease with minimal impact on
function and quality of life that treatment is not required. However, most patients are
significantly impaired by the disorder and need treatment. The mainstays of therapy for CIDP
are intravenous immunoglobulin (IVIG), glucocorticoids, and plasma exchange. (See 'Initial
therapy and ongoing therapy' below and 'Intravenous immune globulin' below and
'Glucocorticoids' below and 'Plasma exchange' below.)
Patients with CIDP should be re-evaluated routinely (eg, at least every three months when
treatments are initiated or changed, and at least twice a year when on maintenance therapy) to
determine whether they are responding to therapy. Maintenance therapy is needed for
responders with active disease. Therapy can be stopped for those who achieve remission, but
relapses are common. (See 'Assessing response to therapy' below and 'Classifying disease
activity' below and 'Duration of therapy' below.)
A different therapy should be substituted for those who fail to respond to the initial mode of
therapy. Alternative immunosuppressant treatment options are available for patients who are
refractory to treatment with IVIG, glucocorticoids, and plasma exchange. (See 'Initial therapy
and ongoing therapy' below and 'Refractory disease' below and 'Other immune modulators'
below.)
Initial therapy and ongoing therapy — For treatment-naïve patients with CIDP who have
active disease and related disability, we recommend initial immune modulating treatment using
either IVIG, glucocorticoids, or plasma exchange. The initial choice among these equally
effective therapies is influenced by disease severity, concurrent illness, venous access,
treatment side effects, availability, and cost [1].
● For patients with severe and fulminant CIDP, we suggest treatment with a rapid immune
modulating therapy such as IVIG, plasma exchange, or pulse high dose glucocorticoids,
rather than standard dose daily glucocorticoids. We prefer initial treatment with IVIG (if
available) or pulse glucocorticoids because they are usually easier to administer than
plasma exchange. (See 'Intravenous immune globulin' below and 'Plasma exchange'
below.)
● For patients with more insidious CIDP, where the goal is to achieve remission, we suggest
initial therapy with glucocorticoids without IVIG or plasma exchange. A glucocorticoid
course of up to eight weeks of pulse-high doses may be required to decide whether there is
a treatment response [2]. Alternative immunosuppressive agents are employed when
glucocorticoids are contraindicated or considered too risky; choices include methotrexate,

cyclosporine, mycophenolate, and azathioprine. However, the effectiveness of these drugs
for CIDP is not established. (See 'Glucocorticoids' below and 'Other immune modulators'
below.)
● For patients who fail to respond to the initial mode of therapy, a different therapy should be
substituted. As an example of treatment substitution, failure to respond to IVIG might
trigger intervention with plasma exchange and/or immunosuppression with glucocorticoids.
The potential yield of this approach is illustrated by a series of patients with CIDP that
included 26 who did not respond to initial therapy [3]. Of these, benefit from an alternative
treatment was observed in nine (35 percent). For 11 patients who required a third
alternative treatment, benefit was observed in three (17 percent).
● A small portion of patients (<20 percent) who respond to the initial therapy may need no
further treatment. However, most patients will relapse sooner or later, and those who
respond to IVIG or plasma exchange may require repeated treatment at intervals that
typically range from two to six weeks. Similarly, relapses are common with glucocorticoid
therapy, particularly when tapering the dose. Thus, for patients with CIDP who relapse after
responding to initial therapy, we suggest repeating the initially successful treatment
modality. For patients on glucocorticoid therapy who relapse, stopping the taper and
increasing the dose is suggested. The timing and dose of ongoing intermittent treatments
should be titrated to avoid relapses.
● For patients initially treated with IVIG who continue to require high doses for many months,
we suggest adding glucocorticoid therapy or an alternative immunosuppressant in the hope
of eventually discontinuing IVIG and achieving clinical remission. Similarly, we suggest
adding glucocorticoid therapy for patients initially treated with plasma exchange who
require frequent treatments over many months.
Suggested doses of IVIG, plasma exchange, and glucocorticoids are discussed in below. (See
'Intravenous immune globulin' below and 'Glucocorticoids' below and 'Plasma exchange' below.)
Assessing response to therapy — The criteria for treatment effect vary per patient. Some
patients may not improve, but the treatment may still be considered effective if the disease
stabilizes without developing progressive deficits. Some abnormalities, due to severe axonal
loss, may be fixed deficits and will not respond to highly effective treatments (see 'Classifying
disease activity' below). It is important for the clinician and patient to be clear on what is likely to
respond and the anticipated timing of the response. In addition, patients and clinicians need to
understand that there may be improvements in energy and well-being that do not necessarily
point to an effect on the disease itself.
Ideally, the success or failure of treatment should be judged using objective methods to quantify
clinical improvement [4,5], such as grip strength dynamometry [6-9] and measures of
impairment such as the Rasch-built Overall Disability Scale (RODS) [10,11], the Overall
Neuropathy Limitations Scale (ONLS) (table 1) [12], or the Inflammatory Neuropathy Cause and
Treatment Disability Scale (INCAT) [13]. These measures have been tested and validated in the
PeriNomS study and have shown to correlate with clinical disease activity. These assessments
should be performed both before and during treatment. We suggest recording the results of a
disability scale such as RODS or ONLS, and a quantitative grip measure (eg, Jamar or
Vigorimeter dynamometer) [7-9]. In addition, the Timed Up and Go (TUG) test [14] or the 10-
meter walking test [15] are simple to do and can be helpful in objectively monitoring disease
progression and treatment effect.
Although such objective measures are not universally employed in clinical practice, there is
accumulating evidence that relying on only the subjective impression of clinical improvement is
associated with continuation of ineffective or inappropriate therapy and even with the over
diagnosis of CIDP [5]. (See "Chronic inflammatory demyelinating polyneuropathy: Etiology,
clinical features, and diagnosis", section on 'Diagnostic pitfalls'.)
Classifying disease activity — The need for continued therapy should be judged by
determining whether CIDP is clinically active or inactive. Towards this end, an expert panel
devised the CIDP Disease Activity Status (CDAS) to classify patients according to disease
activity relative to treatment status (table 2) [16]. The classification is based upon the clinical
assessment of the treating physician. The CDAS classifies patients into five main categories,
with subcategories based mainly upon results of the neurologic examination [16]:
● 1) Cure: ≥5 years off treatment

• A) Normal examination
• B) Abnormal examination, stable/improving
● 2) Remission: <5 years off treatment

● 3) Stable active disease: ≥1 year on treatment

● 4) Improvement: ≥3 months to <1 year on treatment

● 5) Unstable active disease: abnormal examination with progressive or relapsing course

• A) Treatment naïve or <3 months
• B) Off treatment (treatment refractory from prior therapy)
• C) On treatment (worsening despite ongoing therapy)
The CDAS recognizes that some patients have long-term neurologic deficits despite effective
treatment. Such patients would be categorized as 1B, 2B, 3B, or 4B, depending upon treatment
status.
Duration of therapy — In many cases, appropriate treatment of CIDP with IVIG or

glucocorticoids leads to improvement or remission within a few months of starting therapy;
approximately 30 percent of patients will achieve cure or remission [4,16,17]. Therefore,
treatment can be discontinued for patients who achieve sustained remission (eg, 12 months or
longer); though careful follow-up is needed for possible relapses and the need for resuming
therapy.
Refractory disease — For patients with severe CIDP (eg, unstable active disease with a
progressive or relapsing course) who are refractory to treatment with IVIG, glucocorticoids, and
plasma exchange, and are also refractory to glucocorticoids combined with IVIG or plasma
exchange, alternative immunosuppressant treatment options include methotrexate,
cyclosporine, azathioprine, mycophenolate, and cyclophosphamide (see 'Other immune
modulators' below). The choice among these is dependent on a number of factors that include
the expertise of the treating center and clinician, side effect profiles of the alternative
treatments, disease severity, age and sex of patient, and concurrent medical problems. We
recommend that patients who are refractory to initial treatment be evaluated at a center with
particular expertise in CIDP. The GBS/CIDP Foundation International has a number of
designated Centers of Excellence that are available for consultation.
Among these choices, our preferred alternative treatment for patients with no contraindications
is oral methotrexate (7.5 to 15 mg once a week). Patients treated with methotrexate should
receive folic acid 1 mg daily, or folinic acid 2.5 mg weekly, to prevent hematologic and other side
effects. It is unclear whether a response to methotrexate occurs within three or four months or
takes longer. We suggest at least a four month trial, if the clinical condition allows.
For patients with moderately severe disease who do not respond to methotrexate, our preferred
alternative treatment is a trial of cyclosporine. Keeping plasma trough levels between 100 and
150 ng/mL may help ensure a therapeutic response [18,19]. Improvement with cyclosporine
therapy may occur within three months. However, the side effect profile for cyclosporine is
greater than for methotrexate.
Most specialists believe that pulse IV cyclophosphamide can be effective and can lead to long
term remission. The usual dosing is 1000 mg/m2 infused monthly for six months. However, the
risks and side effects must be carefully considered. (See 'Other immune modulators' below.)
Autologous hematopoietic stem cell transplantation (AHSCT) has been used as a treatment of
last resort for patients with CIDP that is refractory to first- and second-line therapies, with
reported benefit in some cases [20,21]. The main risk associated with AHSCT is infection
related to immune suppression. Whether AHSCT is more effective than a lower dose
cyclophosphamide regimen is not known. We urge caution in considering AHSCT and carefully
consider alternatives. (See "Overview of infections following hematopoietic cell
transplantation".)
Pregnancy — The treatment of CIDP in pregnancy is similar to that outlined above; additional
considerations include the following:
● Glucocorticoids are frequently used in pregnancy and are considered safe for the fetus.
● Plasmapheresis is also considered safe for use in pregnancy, although not without potential
complications. (See 'Regimens and side effects of plasma exchange' below.)
● IVIG is generally safe but is associated with potential side effects that might be a particular
concern during pregnancy. (See 'Dose and side effects of IVIG' below.)
The choice among these therapeutic options should be coordinated with the obstetrician. The
risks and benefits of these treatments appear to be finely balanced, and the decision is
influenced by local availability, clinical expertise, and patient preferences.
Other immunosuppressants drugs discussed above are not recommended for use in
pregnancy, particularly early in pregnancy. (See 'Other immune modulators' below.)
PHARMACOLOGIC THERAPY
The mainstays of treatment for CIDP are intravenous immune globulin (IVIG), glucocorticoids,
and plasma exchange [2,22,23]. These treatments appear to be equally effective [13,24,25].
Although data are less compelling, subcutaneous immune globulin (SCIG) therapy may also be
beneficial for CIDP, as reported in a meta-analysis that included mainly small observational
studies [26].
The treatment choice is influenced by patient preference, side effects, treatment cost, duration,
ease of administration, and availability [25]. Advantages and disadvantages of standard
therapies for CIDP include the following [1]:
● IVIG and plasma exchange may lead to a more rapid improvement in CIDP than
glucocorticoid therapy, but are less likely than glucocorticoids to produce a remission.
● IVIG is expensive, and its supply is sometimes limited.
● Glucocorticoids are inexpensive, but chronic use is limited by common and clinically
important side effects.
● Plasma exchange is expensive, invasive, and available only at specialized centers. Venous
access is a major issue.
Intravenous immune globulin — A number of small randomized controlled trials and

systematic reviews have established that IVIG is effective for the short term treatment of CIDP
[6,15,22,25,27-30].
● A systematic review and meta-analysis published in 2013 identified five trials with a total of
235 subjects that compared IVIG with placebo [25]. In addition, the review identified small
trials that compared IVIG with plasma exchange [24], oral prednisolone [13], and
intravenous methylprednisolone [30]. The following observations were noted [25]:
• The rate of disability improvement within one month after treatment was significantly
higher with IVIG than with placebo (relative risk [RR] 2.4, 95% CI 1.72-3.36). To obtain
improvement in one patient, the number needed to treat (NNT) was 3. Overall, IVIG
improved disability for at least two to six weeks.
• The benefit of IVIG was similar to that of plasma exchange, oral prednisolone, and
intravenous methylprednisolone.
● The largest trial included in the meta-analysis was the double-blind multicenter ICE study,
in which 117 patients with CIDP were randomly assigned to treatment with either IVIG or
placebo [6]. IVIG was given as a loading dose of 2 g/kg over two to four days, followed by a
maintenance dosing of 1 g/kg every three weeks for up to 24 weeks in the first treatment
period. The following results were reported:
• The proportion of patients showing clinically meaningful improvement in disability

maintained to week 24, the primary outcome measure, was significantly greater with
IVIG treatment than with placebo (54 versus 21 percent), with an absolute difference of
33.5 percent (95% CI 15.4-51.7).
• During a 24 week extension phase, 57 patients who were first-period IVIG responders
were randomly reassigned to IVIG or placebo. Patients who continued to receive IVIG
had a significantly longer time to relapse than those reassigned to placebo during this
phase, and the relapse rate was significantly lower for patients treated with IVIG (13
versus 45 percent, hazard ratio 0.19, 95% CI 0.05-0.70).
Thus, the ICE study confirmed evidence from earlier trials that IVIG is effective for the
treatment of CIDP. In addition, the ICE results suggested that the benefit of IVIG extends to
as long as 48 weeks with maintenance treatments of 1 g/kg every three weeks [6]. As a
result of these findings, the IVIG formulation used in this trial became the first FDA-
approved treatment for CIDP. However, it should be understood that there is no known
difference in effectiveness between different IVIG preparations.
While IVIG therapy can usually control CIDP, most patients require repeated expensive
treatments every two to six weeks for many years, since IVIG monotherapy does not usually
lead to remission. This point is illustrated by a 20-year review of 95 patients treated with IVIG
for CIDP [31]. More than 75 percent of the patients improved, but of those who improved, over
85 percent required repeated treatment, while fewer than 15 percent were able to discontinue
treatment at a mean of 3.5 years and median of 2.1 years. Severity at onset and residual deficit
were negative predictors of discontinuation. However, the placebo effect in the ICE trial, as well
as in the methotrexate trial, suggests that the number of patients who remain stable after
discontinuation of IVIG may be significantly greater than 15 percent.
IVIG should be used cautiously in patients with severe kidney disease because of its
association with renal failure [1]. Only formulations without sucrose and with low osmolality
should be considered.
Dose and side effects of IVIG — The initial dose of IVIG is 2 g/kg infused over two to five
days (eg, 0.4 g/kg per day for five days). Observation should determine if the treatment is
effective. Benefit may not be apparent after the first course of IVIG treatment, and some experts
advocate repeating infusion of IVIG (1 g/kg) every three weeks for two to three months after the
initial treatment before determining efficacy [29]. Others advocate a slightly more frequent
dosing (1 g/kg every two weeks for two to three months) before determining efficacy.
Many patients with CIDP require repeat IVIG maintenance dosing every two to six weeks. For
patients who respond well to initial treatment and remain clinically stable, the maintenance dose
can be tapered to 1 g/kg, or as low as 0.4 g/kg per dose, given over one to two days. The taper
of IVIG dose and/or frequency should be adjusted so that the patient does not deteriorate
between doses.
Side effects of IVIG include headache, nausea, fever, aseptic meningitis, rash, acute renal
failure (mostly related to sucrose containing products), and rarely hyperviscosity. IgA deficiency
can lead to anaphylaxis in patients treated with IVIG.
Hematologic side effects (eg, hemolysis, neutropenia) of IVIG are reviewed in detail elsewhere.
(See "Intravenous immune globulin: Adverse effects", section on 'Hematologic complications'.)
Nonhematologic side effects of IVIG (eg, anaphylaxis, renal toxicity, aseptic meningitis) as well
as the risk of thrombosis are discussed in depth separately. (See "Immune globulin therapy in
primary immunodeficiency".)
Glucocorticoids — The benefit of glucocorticoids for CIDP was first reported in the 1950s [32],
but no large controlled trials have been performed.
● In a small trial, 14 patients with CIDP and no prior treatment completed a three-month
regimen of oral prednisone, beginning with a dose of 120 mg every other day during the
first week, followed by a slow taper off prednisone by the end of 12 weeks [33]. Patients
who received prednisone had a clinically meaningful improvement compared with 14
patients assigned to placebo. This was the only trial comparing glucocorticoids with no
treatment that was eligible for inclusion in a systematic review, which concluded that it
provided very low quality evidence of a beneficial effect of oral glucocorticoids for CIDP
[34].
● In a multicenter crossover trial of 32 patients with CIDP, oral prednisolone (60 mg daily for
two weeks, tapered down to 10 mg daily over one month) was compared with IVIG (2 g/kg
administered over one to two days) [13]. Both treatments were associated with a significant
improvement in disability, and there was no significant difference between the two
treatment groups.
● Substantial data from retrospective series suggest that oral glucocorticoids are beneficial
for CIDP [35-39]. The maximum benefit of glucocorticoid therapy in these studies was seen
after one to six months of treatment [1]. However, relapses were common, particularly
when tapering the dose [37].
● Clinical experience suggests that glucocorticoid therapy is more likely to produce a clinical
remission than either IVIG or plasma exchange.
The dosing of glucocorticoids is not agreed upon. There are several equally reasonable ways to
start and then taper the dose. Regimens including daily and alternate day oral prednisone,
weekly pulse oral methylprednisolone, and weekly or monthly pulse intravenous
methylprednisolone. How fast to taper and when to taper depend on a variety of factors.
However, there is increasing use of intravenous or oral pulse glucocorticoids rather than daily
oral prednisone [40], and we prefer the use of pulse glucocorticoids, either oral or intravenous,
rather than daily oral prednisone, for initiation and titration of dosing. Pulse dosing may have
both a greater chance for early efficacy and a lower side effect profile.
Chronic glucocorticoid use is limited by common and clinically important side effects that
include weight gain, cushingoid appearance, easy bruising and skin fragility, cataracts, aseptic
necrosis of the femoral or humeral heads, hypertension, diabetes, and osteoporosis. Thus,
glucocorticoid therapy is generally contraindicated in patients with peptic ulcer disease, brittle
diabetes, refractory hypertension, severe osteoporosis, and systemic fungal infections [1]. (See
"Major side effects of systemic glucocorticoids".)
Oral — Whether to use daily, alternate-day, or pulsed oral glucocorticoids is determined by

the individual nature of the patient's medical health and the severity and pattern of CIDP.
In the PREDICT study, 40 patients with newly diagnosed, treatment-naive CIDP were randomly
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assigned to treatment with either high-dose pulsed oral dexamethasone (40 mg daily for four
days followed by placebo for 24 days, repeated for six cycles) or daily prednisolone (starting
with 60 mg daily for five weeks and tapering to alternate day doses and then to zero over the
following 27 weeks) [40]. For the primary endpoint, the remission rate at 12 months, there was
no significant difference between the pulsed high-dose dexamethasone and standard
prednisolone treatment groups (10 of 24 [42 percent] versus 6 of 16 [38 percent], odds ratio
1.19, 95% CI 0.3-4.4). While this study did not show an advantage for pulsed dexamethasone, it
does provide an alternative regimen for giving glucocorticoids that may be useful for some
patients. Some practitioners believe that weight gain and cushingoid side effects are less with
pulsed glucocorticoid treatment, while sleep and psychologic effects may be greater.
Although less well-studied, retrospective evidence suggests that weekly pulse

methylprednisolone (500 mg once a week) is also an effective option for long-term treatment of
CIDP [41].
Alternate-day glucocorticoid therapy may reduce the incidence of side effects, but some experts
believe it is less effective than a daily dosing regimen [1]. Daily dosing may be preferred for
younger patients and those patients with little in the way of side effects. In addition, daily dosing
may be preferred in patients with diabetes, so that there is a more even daily effect on blood
sugars.
One suggested oral regimen is to start prednisone at 1 to 1.5 mg/kg daily (usually around 50 to
80 mg daily; no more than 100 mg). The dose is then titrated according to clinical response.
Patients who have a good response can begin dose tapering after one to three months.
Glucocorticoid tapering can be done in many ways and should be individualized as a daily or
every other day regimen. The dose can be tapered by 5 to 10 mg every two to four weeks in
clinically stable patients.
As an example, with the every other day regimen, the taper from 80 mg daily would begin with
80 mg alternating with 60 mg, then a month later to 80 mg alternating with 40 mg, and
eventually to 80 mg alternating with 0 mg. Tapering of the high-dose day would then
commence. We suggest slowing down the rate of the taper once a dose of 30 mg every other
day (or 15 mg daily) is reached. It is usually necessary to go back up to a higher dose by at
least 10 to 20 mg if symptoms recur during the taper.
Intravenous — As with oral glucocorticoid therapy, there is no standard regimen for pulse
intravenous (IV) glucocorticoids. One suggested regimen is an initial dose of IV

methylprednisolone (1000 mg/day) for three days, followed by 1000 mg one day a week for four
weeks [42]. Tapering is accomplished by slowly decreasing the frequency of dosing to once
every 2 to 12 weeks.
Limited observational data suggest that IV and oral glucocorticoid regimens are equally
effective, but that IV therapy is associated with less weight gain and fewer cushingoid features,
but possibly more restlessness and sleep problems [42]. The dreaded risks of avascular
necrosis, gastrointestinal hemorrhage, and osteoporotic bone fracture are not known to be
different between the different regimens. One advantage of high dose pulse treatment is that
the effect may be seen more quickly than with the daily oral prednisone.
Plasma exchange — Two small randomized clinical trials have found that plasma exchange is
effective for the short term treatment of CIDP [43,44]. These trials were the only ones identified
as eligible in a systematic review and meta-analysis of plasma exchange for the treatment of
CIDP [45].
In the meta-analysis, the following observations were reported [45]:
● There was significantly more improvement (using an impairment scale score) during or
after plasma exchange than after sham exchange
● Overall, about two-thirds of patients initially improved with plasma exchange
● In one of the included trials [44], relapses of CIDP occurred in 8 of 12 patients (67 percent)
who initially responded to plasma exchange, and in seven of these patients, the relapse
occurred within a week or two after stopping plasma exchange
A number of observational studies have also found that plasma exchange is effective for CIDP
[3,46-51].
Like IVIG, treatment with plasma exchange alone is unlikely to lead to remission, and plasma
exchange has the added concerns of an invasive procedure that requires repeated venous
access and (often) indwelling catheters that are susceptible to clotting and infection. In addition,
plasma exchange is available only at specialized centers.
Regimens and side effects of plasma exchange — For patients with CIDP and severe
disability, we suggest plasma exchange beginning with four to six exchanges over 8 to 10 days.
The plasma exchange schedule after that depends on clinical response, but can usually be
decreased to one exchange every three to four weeks. However, if symptoms occur between
treatments, the schedule would be modified to eliminate the interval changes. The
implementation of therapeutic plasma exchange, including techniques and regimens, is
discussed in detail separately. (See "Therapeutic apheresis (plasma exchange or cytapheresis):
Indications and technology".)
The main complications of plasma exchange are hypotension, sepsis, and problems with
intravenous access. (See "Therapeutic apheresis (plasma exchange or cytapheresis):
Complications".)
Other immune modulators — New CIDP therapies are needed that are more effective, more
specific, more cost effective, and less toxic than currently available treatments (ie, IVIG,
glucocorticoids, and plasma exchange).
A number of immunosuppressive agents have been reported to be beneficial for CIDP in

observational studies, but none have been rigorously studied in randomized controlled trials
with enough power to provide convincing evidence of effectiveness [52].
The list of immune modulator drugs that have been used to treat CIDP includes the following:
● Azathioprine [38,39,53-55]
● Cyclophosphamide [39,56-60]
● Cyclosporine [18,19,61,62]
● Etanercept [63]
● Interferon alpha-2a [64,65]
● Interferon beta-1a [66,67]
● Mycophenolate mofetil [68-70]
● Methotrexate [71-73]
● Rituximab [74-78]
● Tacrolimus [79]
While many of these agents remain promising, evidence from larger clinical trials is needed to
determine whether they are effective for CIDP. Of note, a small randomized placebo-controlled
trial in patients with CIDP requiring intravenous immune globulin or glucocorticoids found no
significant benefit for oral methotrexate (15 mg weekly) on any of the primary or secondary
outcome measures [72]. However, there was a remarkably high placebo effect suggesting that
the design of the trial may have contributed to the inability to find benefit from the drug. Thus,
the role of methotrexate in CIDP remains unproven.
Historically, azathioprine and cyclophosphamide have probably been the most commonly used
drugs for CIDP from this list. Azathioprine is generally used as a steroid sparing medication or
reserved for patients with mild disease; a therapeutic response may take over six months. An
alternative is mycophenolate mofetil, and clinical improvement with this agent may also take a
long time.
Some [39,56,57,59,60] but not all [58] studies have found that cyclophosphamide may be
effective for CIDP; a systematic review concluded that the available observational studies
suggest, but do not prove, that cyclophosphamide treatment is beneficial [52]. These studies
included intravenous pulse regimens and oral daily regimens of cyclophosphamide for patients
who failed other therapies. Some of the responses were quite remarkable and sustained [60].
Because of this, cyclophosphamide has been advocated, despite its potential toxicity, for
patients with severe disease refractory to other treatments [60], and high-dose
cyclophosphamide therapy has used in attempt to reset the immune system [80,81]. Both
moderate (1000 mg/m2 monthly for six months) and very high-dose (eg, 50 mg/kg administered
over one hour intravenously for four days) cyclophosphamide regimens may induce a remission
of the disease. However, cyclophosphamide treatment is associated with potentially life-
threatening side effects and should therefore be carefully considered and performed only at
specialized centers.
The role of B cell depletions therapies such as rituximab in CIDP is undefined. However,
subgroups of CIDP associated with IgG4 antibodies to neurofascin or contactin may be
refractory to IVIG and glucocorticoids but responsive to rituximab [82-84].
PROGNOSIS
Approximately two-thirds of patients with CIDP will initially respond to any single standard
therapy (intravenous immune globulin [IVIG], glucocorticoid, and plasma exchange), while
about 10 to 15 percent of patients are resistant to all of these. However, data are limited
regarding the long-term prognosis of CIDP.
In a retrospective review of 106 patients with a consensus diagnosis of CIDP and a mean
follow-up of 6.4 years who were blindly classified by experts using the CIDP Disease Activity
Status (CDAS) (see 'Classifying disease activity' above), the proportion of patients in each
category was as follows (table 2) [16]:
● Cure (five or more years off treatment), 11 percent

● Remission (less than five years off treatment), 20 percent
● Stable active disease (one or more years on treatment), 44 percent
● Improving (three months to less than one year on treatment), 7 percent
● Unstable active disease, 18 percent
These and other data suggest that approximately 30 percent of patients with CIDP will achieve
cure or remission [16,17].
An earlier study examining long-term outcome reviewed 38 Japanese patients with CIDP who
had at least five years of follow-up [85]. These patients had been treated with various regimens,
including glucocorticoids (89 percent), IVIG (45 percent), and plasma exchange (34 percent).
The following observations were reported [85]:
● Complete remission, defined as lasting more than two years with normal nerve conduction
studies, was noted in 10 patients (26 percent).
● Partial remission, defined as able to walk, was noted in 23 (61 percent). In this group, there
were 13 patients who had stopped immune modulating treatments, and 10 patients who
required intermittent or continuous therapy, mainly glucocorticoids.
● Severe disability (unable to walk) or relapses related to tapering of medication were

present in five (13 percent).
SUMMARY AND RECOMMENDATIONS
● For patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who have
clinically significant disability, we recommend immune modulating treatment using either
intravenous immune globulin (IVIG), glucocorticoids, or plasma exchange (Grade 1B).
(See 'Pharmacologic therapy' above and 'Intravenous immune globulin' above and
'Glucocorticoids' above and 'Plasma exchange' above.)
● For patients with severe and fulminant CIDP, we suggest treatment with a rapid immune
modulating therapy such as IVIG, plasma exchange, or pulse high dose glucocorticoids,
rather than standard dose daily glucocorticoids (Grade 2C). We prefer initial treatment with
IVIG (if available) or pulse glucocorticoids because they are usually easier to administer
than plasma exchange. For patients with more insidious CIDP, where the goal is to achieve
remission, we suggest initial therapy with glucocorticoids with or without IVIG or plasma
exchange (Grade 2C). (See 'Approach to treatment' above.)
● For patients who fail to respond to the initial mode of therapy, we would substitute an
alternative treatment. As an example, failure to respond to IVIG triggers intervention with
plasma exchange or glucocorticoids. (See 'Approach to treatment' above.)
● Patients with CIDP should be re-evaluated routinely to determine whether they are
responding to therapy. Maintenance therapy is needed for responders with active disease.
Therapy can be stopped for those who achieve remission, but relapses are common. (See
'Assessing response to therapy' above and 'Classifying disease activity' above and
'Duration of therapy' above.)
● Most patients with CIDP who respond to initial IVIG or plasmapheresis therapy will relapse
when the therapy is discontinued and will require treatment. For such patients, we retreat
and suggest repeating the initially successful treatment modality (Grade 2C). For patients
on glucocorticoid therapy who relapse, stopping the taper and/or increasing the dose is
reasonable. The timing and dose of ongoing intermittent treatments should be titrated to
avoid relapses. (See 'Approach to treatment' above.)
● For patients with CIDP initially treated with IVIG who require high dose therapy for many
months, we suggest adding glucocorticoid therapy (Grade 2C). Similarly, we suggest
adding glucocorticoid therapy for patients initially treated with plasma exchange who
require frequent treatments over many months (Grade 2C). (See 'Approach to treatment'
above.)
● For patients with severe CIDP who are refractory to treatment with IVIG, glucocorticoids,
and plasma exchange, and are also refractory to glucocorticoids combined with IVIG or
plasma exchange, or have unacceptable side effects with these regimens, we suggest
treatment with an alternative immunosuppressant (Grade 2C). Options include
methotrexate, cyclosporine, azathioprine, mycophenolate, or cyclophosphamide. The
choice among these agents must be individualized. Patients should be informed that the
benefit of these therapies for CIDP is unproven, and that these treatments are associated
with a risk of serious side effects. (See 'Other immune modulators' above and 'Refractory
disease' above.)
● The prognosis of CIDP is generally favorable, but data are limited. Approximately 30
percent of patients achieve cure or long-term remission, while approximately 18 percent
have unstable active disease with a progressive or relapsing course. (See 'Prognosis'
above.)
Use of UpToDate is subject to the Subscription and License Agreement.
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Topic 5265 Version 27.0
GRAPHICS
Overall Neuropathy Limitations Scale (ONLS)
Name:
Date:
Instructions: The examiner should question and observe the patient in order to determine the answers to the
following questions. Note should be made of any other disorder other than peripheral neuropathy which limits
function at the foot of the page.
ARM SCALE
Does the patient have any symptoms in their hands or arms, eg, Yes No
tingling, numbness or weakness?
(if "no", please go to "legs"
section)
Is the patient affected in their ability to: Not affected Affected but Prevented
not
prevented
Wash and brush their hair
Turn a key in a lock
Use a knife and fork together (or spoon, if knife and

fork not used)
Do or undo buttons or zips
Dress the upper part of their body excluding buttons

or zips
If all these functions are prevented can the patient Yes No Not applicable
make purposeful movements with their hands or
arms?
Arm grade
0 = Normal
1 = Minor symptoms in one or both arms but not affecting any of the functions listed
2 = Disability in one or both arms affecting but not preventing any of the functions listed
3 = Disability in one or both arms preventing at least one but not all functions listed
4 = Disability in both arms preventing all functions listed but purposeful movement still possible
5 = Disability in both arms preventing all purposeful movements
SCORE = _____
LEG SCALE
Yes No Not
applicable
Does the patient have difficulty running or climbing

stairs?
Does the patient have difficulty with walking?
Does their gait look abnormal?
How do they mobilize for about 10 meters (ie, 33 feet)?
Without aid
With one stick or crutch or holding to someone's

arm
With two sticks or crutches or one stick or crutch

holding onto someone's arm or frame
With a wheelchair
If they use a wheelchair, can they stand and walk 1

meter with the help of one person?
If they cannot walk as above are they able to make

some purposeful movements of their legs, eg,
reposition legs in bed?
Does the patient use ankle foot

orthoses/braces
orthoses/braces? (please circle)
If yes: (please circle) right/left
Leg grade
0 = Walking/climbing stairs/running not affected
1 = Walking/climbing stairs/running is affected, but gait does not look abnormal
2 = Walks independently but gait looks abnormal
3 = Requires unilateral support to walk 10 meters (stick, single crutch, one arm)
4 = Requires bilateral support to walk 10 meters (sticks, crutches, crutch and arm, frame)
5 = Requires wheelchair to travel 10 meters but able to stand and walk 1 meter with the help of one person
6 = Restricted to wheelchair, unable to stand and walk 1 meter with the help of one person, but able to make
some purposeful leg movements
7 = Restricted to wheelchair or bed most of the day, unable to make any purposeful movements of the legs
SCORE = _____
Overall Neuropathy Limitation Scale = arm scale (range 0 to 5) + leg scale (range 0 to 7); (range: TOTAL
0 (no disability) to 12 (maximum disability)) SCORE =
_____
Is there any disorder, other than peripheral neuropathy, which Yes No

affects the above functions?
If yes please describe:
From: Graham RC, Hughes RA. A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol
Neurosurg Psychiatry 2006; 77:973. Reproduced with permission from BMJ Publishing Group Ltd. Copyright © 2006.
Graphic 105872 Version 2.0
CDAS classification scheme and proportion of CIDP patients within each category
Patients (n = 106)
1. Cure: ≥5 years off treatment 11% cure
A. Normal examination 6 (6%)
B. Abnormal examination, stable/improving 5 (5%)
2. Remission: <5 years off treatment 20% remission
3. Stable active disease: ≥1 year, on treatment 44% stable disease
4. Improvement: ≥3 months <1 year, on treatment 7% improving
5. Unstable active disease: Abnormal examination with progressive 18% active disease
or relapsing course
course*
A. Treatment naïve or <3 months 6 (6%)
B. Off treatment 7 (7%)
C. On treatment 5 (5%)
The classification is based upon the clinical assessment of the treating physician at the time of last evaluation.
CIDP: chronic inflammatory demyelinating polyneuropathy; CDAS: CIDP Disease Activity Status.
* 5B and 5C refer to patients who were treatment refractory from prior therapy or worsening despite ongoing therapy.
From: Gorson KC, van Schaik IN, Merkies IS, et al. Chronic inflammatory demyelinating polyneuropathy disease activity
status: recommendations for clinical research standards and use in clinical practice. J Peripher Nerv Syst 2010; 15:326.
DOI: 10.1111/j.1529-8027.2010.00284.x. Copyright © 2010 Peripheral Nerve Society. Reproduced with permission of
John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can
be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email:
permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this
article on Wiley Online Library (http://onlinelibrary.wiley.com).
Graphic 104439 Version 1.0
Contributor Disclosures
Richard A Lewis, MD Consultant/Advisory Shire; CSL Behring; Pharnext; Biotest [CIDP (IVIg)]; Axelacare
[CIDP (home infusion IVIg)]; NuFactor [Editorial board]; Akcea; Alnylam; Alexion; Argenx. Employment:
Premier Pharmacy Services [Medical Director]. Jeremy M Shefner, MD, PhD Grant/Research Support:
Biogen Idec; Cytokinetics; Amylyx; Biohaven; Orphazyme; Brainstorm [ALS]; Biotie [Parkinson disease].
Consultant/Advisory Boards: Cytokinetics; Mitsubishi Tanabe; Biohaven; Pharnext; AveXis; Biogen Idec
[ALS]; MT Pharma [ALS (Edaravone)]. John F Dashe, MD, PhD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.
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Chronic inflammatory demyelinating polyneuropathy: Treatment and prognosis - UpToDate 25/04/2019, 16+40

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Chronic inflammatory demyelinating polyneuropathy:

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an entity that describes a

Of major importance, CIDP generally responds to immunomodulatory treatment with

glucocorticoids are contraindicated or considered too risky; choices include methotrexate,

● 1) Cure: ≥5 years off treatment

● 2) Remission: <5 years off treatment

● 3) Stable active disease: ≥1 year on treatment

● 4) Improvement: ≥3 months to <1 year on treatment

● 5) Unstable active disease: abnormal examination with progressive or relapsing course

Duration of therapy — In many cases, appropriate treatment of CIDP with IVIG or

● IVIG is expensive, and its supply is sometimes limited.

Intravenous immune globulin — A number of small randomized controlled trials and

improved disability for at least two to six weeks.

• The proportion of patients showing clinically meaningful improvement in disability

discontinuation of IVIG may be significantly greater than 15 percent.

Oral — Whether to use daily, alternate-day, or pulsed oral glucocorticoids is determined by

Although less well-studied, retrospective evidence suggests that weekly pulse

intravenous (IV) glucocorticoids. One suggested regimen is an initial dose of IV

In the meta-analysis, the following observations were reported [45]:

● Overall, about two-thirds of patients initially improved with plasma exchange

A number of immunosuppressive agents have been reported to be beneficial for CIDP in

● Cure (five or more years off treatment), 11 percent

The following observations were reported [85]:

● Severe disability (unable to walk) or relapses related to tapering of medication were

SUMMARY AND RECOMMENDATIONS

Use of UpToDate is subject to the Subscription and License Agreement.

1. Ropper AH. Current treatments for CIDP. Neurology 2003; 60:S16.

3. Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy:

9. Rajabally YA, Narasimhan M. Jamar hand-held grip dynamometry in chronic inflammatory

13. Hughes R, Bensa S, Willison H, et al. Randomized controlled trial of intravenous

19. Odaka M, Tatsumoto M, Susuki K, et al. Intractable chronic inflammatory demyelinating

20. Press R, Askmark H, Svenningsson A, et al. Autologous haematopoietic stem cell

21. Kazmi MA, Mahdi-Rogers M, Sanvito L. Chronic inflammatory demyelinating

22. Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous

cross-over study. Brain 1996; 119 ( Pt 4):1067.

30. Nobile-Orazio E, Cocito D, Jann S, et al. Intravenous immunoglobulin versus intravenous

35. DeVivo DC, Engel WK. Remarkable recovery of a steroid-responsive recurrent

36. Oh SJ. Subacute demyelinating polyneuropathy responding to corticosteroid treatment.

37. Wertman E, Argov Z, Abrmasky O. Chronic inflammatory demyelinating

42. Lopate G, Pestronk A, Al-Lozi M. Treatment of chronic inflammatory demyelinating

55. Monaco S, Turri E, Zanusso G, Maistrello B. Treatment of inflammatory and

59. Brannagan TH 3rd, Pradhan A, Heiman-Patterson T, et al. High-dose cyclophosphamide

65. Pavesi G, Cattaneo L, Marbini A, et al. Long-term efficacy of interferon-alpha in chronic

68. Umapathi T, Hughes R. Mycophenolate in treatment-resistant inflammatory neuropathies.

70. Bedi G, Brown A, Tong T, Sharma KR. Chronic inflammatory demyelinating

73. Díaz-Manera J, Rojas-García R, Gallardo E, Illa I. Response to methotrexate in a chronic

74. Knecht H, Baumberger M, Tobòn A, Steck A. Sustained remission of CIDP associated

76. Gono T, Matsuda M, Shimojima Y, et al. Rituximab therapy in chronic inflammatory

78. Münch C, Anagnostou P, Meyer R, Haas J. Rituximab in chronic inflammatory

79. Ahlmén J, Andersen O, Hallgren G, Peilot B. Positive effects of tacrolimus in a case of

82. Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Antibodies to contactin-1 in chronic

83. Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Neurofascin IgG4 antibodies in CIDP

84. Querol L, Rojas-García R, Diaz-Manera J, et al. Rituximab in treatment-resistant CIDP

Topic 5265 Version 27.0

Overall Neuropathy Limitations Scale (ONLS)

Wash and brush their hair

Turn a key in a lock

Use a knife and fork together (or spoon, if knife and

Do or undo buttons or zips

Dress the upper part of their body excluding buttons

5 = Disability in both arms preventing all purposeful movements

Does the patient have difficulty running or climbing