596

Chronic Inflammatory Demyelinating

Polyradiculoneuropathy
Jeffrey Shije, MD1 Thomas H. Brannagan III, MD1

1 Peripheral Neuropathy Center, Neurological Institute, Columbia Address for correspondence Thomas H. Brannagan, III, MD, Peripheral
University, New York, New York Neuropathy Center, Neurological Institute, Columbia University, New York,
NY 10032 (e-mail: Tb2325@cumc.columbia.edu).
Semin Neurol 2019;39:596–607.

Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a relatively

common autoimmune disorder affecting the peripheral nerves and nerve roots, often
causing progressive or recurrent weakness with diminished reflexes. Electrodiagnostic
(EDx) studies, cerebral spinal fluid (CSF) analysis, and nerve biopsy may help provide

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supportive evidence for the diagnosis. Most cases have a favorable response to one of
the three first-line treatments: corticosteroids, IV immunoglobulin (IVIG) and plasma-
Keywords pheresis. Responses to these treatments may vary among individual patients. There is
► chronic inflammatory evidence that a small percentage of CIDP patients with IgG class 4 (IgG4) auto-
demyelinating antibodies to paranodal proteins have characteristic clinical features and poorer
polyradiculoneuro- response to IVIG. Chemotherapy and other immunomodulatory agents, as well as
pathy hematopoietic stem cell transplantation, may be considered in refractory cases.
► CIDP The degree of disability varies, and most patients require ongoing treatment to
► inflammatory maintain stable disease, although long-term remission or cure may be achieved in
neuropathy some patients.

It had been recognized as early as the 1950s that some
patients with recurrent polyneuropathy would have symp-
tom improvement from corticosteroids.1 Subsequently, Dyck
et al2 demonstrated histological features of nerve inflamma-
tion and chronic demyelination in a group of patients with
progressive or recurrent weakness, and they had a favorable
response to corticosteroid. They were initially referred to as
chronic inflammatory polyradiculoneuropathy. We now
refer to these cases as chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP).
Clinical Presentation
The clinical features of typical CIDP are summarized
in ►Table 1. The progressive or recurrent weakness in typical
CIDP is relatively symmetrical, often involving distal and
proximal extremities. Absent or diminished reflexes are seen
in nearly all cases. There are accompanying sensory symp-
toms, such as numbness and paresthesias, in the majority of
the cases, although pain is less frequent.2–5 Younger patients
are more likely to have relapsing–remitting disease, whereas
older patients are more likely to have a progressive course.5
Ataxia and coarse tremor may be seen, presumably due to
severe sensory/proprioceptive impairment. Of note, ataxia
and tremor have been reported more frequently in CIDP
patients with autoantibodies against paranodal proteins
such as neurofascin-155 and contactin-1.6–11
Cranial neuropathies, and respiratory and/or autonomic
dysfunction may be present, although they are infrequent.
Cranial nerve involvement, including oculomotor, facial, or
bulbar, have been reported in 7% to 16% of cases.2–5 Respira-
tory failure is rare in CIDP, although it has been reported.12
One study reported prolonged phrenic nerve latency in most
of the asymptomatic CIDP patients, although abnormal
pulmonary function tests were more associated with
reduced phrenic nerve compound motor action potential
(CMAP) amplitude, which was rare.13 Autonomic symptoms
are infrequent and generally mild when present. These may
include bowel and bladder complaints, while dry mouth/
eyes, orthostatic intolerance, sexual dysfunction, flushing,
and hyperhidrosis are even rarer. Subclinical abnormalities
on autonomic testing may be seen, but there is no correlation

Issue Theme Peripheral Neuropathies; Copyright © 2019 by Thieme Medical DOI https://doi.org/
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Table 1 Clinical features of typical CIDP
Symptoms Frequency of symptoms
Weakness All
Areflexia/hyporeflexia Nearly all
Sensory symptoms Majority
Tremor and ataxia, presumably
related to severe sensory/
proprioceptive impairment
Cranial nerve involvement Infrequent, 7%–16%
Respiratory symptoms Infrequent (respiratory failure is
rare)
Autonomic symptoms Infrequent
between autonomic dysfunction and the severity of the
overall disease.14–18
Some CIDP patients have an acute-onset presentation that
is clinically indistinguishable from acute inflammatory
demyelinating polyradiculoneuropathy (AIDP) or Guillain–
Barre Syndrome (GBS) early in the disease course. One
prospective cohort study showed that GBS patients had no
more than two treatment-related fluctuations and no pro-
gression of their neurological impairment beyond
two months.19 Therefore, if a patient was previously thought
to have GBS but continues to worsen two months after
symptom onset, it would raise the suspicion of CIDP instead.
Epidemiology
The epidemiologic data varies depending on geography. CIDP
prevalence ranging from 0.8/100,000 (Tottori Prefecture,
Japan) to 8.9/100,000 (Olmsted County, Minnesota) have
been reported.20–24 The annual incidence may range from
0.15 to 1.6/100,000.20,25 The different criteria used to define
the disease may contribute to the variation in its reported
prevalence and incidence.26 CIDP can affect age groups from
childhood to beyond the eighth decade of life. The highest
prevalence reported is in the seventh decade of life, although
the mean age of onset may be around the fourth to fifth
decade of life; men are more likely to be affected than
women.20,25,27 It had been reported that the disease onset
may be preceded by infection or immunization in up to one
third of CIDP cases, which is notably less compared with GBS,
where nearly half may report an antecedent infection.3,28
Pathophysiology
There is evidence that CIDP is an autoimmune demyelinating
disorder of the peripheral nerves and nerve roots. Nerve
biopsies have demonstrated chronic segmental demyelination
CIDP Shije and Brannagan 597
Other characteristics
Relatively symmetrical, involving proximal and
distal extremities. May be progressive or
relapsing–remitting.
Markedly diminished if not absent
Numbness and/or paresthesia. Pain is relatively less
frequent.
May be associated with paranodal antibody (i.e.,
neurofascin-155, contactin-1, etc.)
Oculomotor, facial or bulbar involvement all have
been reported
Subclinical prolonged phrenic nerve latency may
be present, but abnormal phrenic nerve CMAP
amplitude is more associated with abnormal PFT,
which is rarer.
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Typically mild even if present, i.e., bowel/bladder
symptoms. Sicca syndrome, orthostatic
symptoms, and sexual dysfunction are even rarer.
and remyelination, as well as perivascular and endoneurial
infiltration by macrophages and T cells.2,29,30 Elevated levels of
proinflammatory cytokines associated with cell-mediated
immune response have been reported in the serum, CSF, and
nerve biopsies of CIDP patients during active and subacute
disease.31–35 In an animal model, a spontaneous autoimmune
peripheral polyneuropathy (SAPP), similar to CIDP in patho-
physiology, can be produced in mice from unopposed cell-
mediated immunity.36 The exact antigenic trigger is not
identified in most cases of CIDP. Immunization with peripheral
myelin proteins (i.e., P0, P2, and PMP22) may produce a chronic
experimental allergic neuritis (EAN) in mice and rabbits,37,38
and anti-P0 antibodies from CIDP patients may cause demye-
linating neuropathy when injected into experimental ani-
mals.39 However, antimyelin protein antibodies have only
been reported in a minority of CIDP patients, and these
autoantibodies have not elicited consistent inflammatory
responses in animals.40–43 More recently, autoantibodies
against paranodal proteins, that is, neurofascin-155 and con-
tactin-1, have been reported in CIDP cases with characteristic
clinical features and responses to treatment, but these only
account for 2% to 6% of cases.44
Diagnosis
CIDP may be suspected in someone with progressive or
recurrent weakness in the proximal and distal extremities
for at least two months. Albuminocytologic dissociation
(elevated CSF protein without CSF pleocytosis) is often
present. Aside from nerve biopsy, evidence of nerve demye-
lination may be demonstrated by electrodiagnostic (EDx)
testing. The Edx features of demyelination include significant
reduction of motor conduction velocities, presence of con-
duction block and temporal dispersion, significant prolonga-
tion of F-wave latencies, distal motor latencies and distal
compound motor action potential (CMAP) duration.
Seminars in Neurology Vol. 39 No. 5/2019
598 CIDP Shije and Brannagan
Various clinical and EDx criteria for CIDP had been proposed
by different investigators early on,2,4,45 including a research
criteria developed by an ad hoc subcommittee of the American
Academy of Neurology (AAN) AIDS Task Force (►Table 2).46
The AAN research criteria required three of its four EDx
parameters of demyelination (abnormal conduction velocity,
distal motor latency, F-wave latency, and conduction block/
temporal dispersion) to be present, with each of those (except
conduction block/temporal dispersion) in at least two nerves,
plus nerve biopsy and CSF analysis, for a definite diagnosis of
CIDP. It had been pointed out that the AAN research criteria
may be too stringent and may not be met by many patients
with CIDP.47,48 Also, it is not uncommon for the neuropathy in
CIDP to have substantial secondary axonal loss so that EDx
evaluation of demyelination is no longer feasible.49 There have
been numerous proposed modifications to the diagnostic
criteria in the interest of improving its sensitivity and speci-
ficity. For example, Saperstein et al48 required only two of the
four EDx parameters of demyelination and did not mandate
nerve biopsy to promote diagnostic sensitivity, while having
more stringent EDx requirements for conduction block to
promote specificity. Nicolas et al50 placed more emphasis on
conduction block and temporal dispersion to promote diag-
nostic distinction from other demyelinating neuropathies.
This emphasis on conduction block/temporal dispersion was
also reflected in the criteria by the Inflammatory Neuropathy
Cause and Treatment (INCAT) group (►Table 3),51 which has
been used in large clinical trials. Patients may be diagnosed or
enrolled in clinical trials more promptly based on the INCAT
Table 2 Features of the AAN research criteria for CIDP46
Clinical features:
Progressive or relapsing motor and sensory dysfunction of
more than one limb for >2 months.
Reduced or absent reflexes
3 of the 4 EDx parameters below are required:
a. Reduced conduction velocity <80% of LLN if
amplitude > 80% of LLN or <70% of LLN if amplitude
<80% of LLN in 2 nerves
b. Prolonged motor distal latency >125% of ULN if amplitude
>80% of LLN or >150% of ULN if amplitude <80% of LLN in
2 nerves
c. Prolonged F-wave latency >120% of ULN if amplitude
>80% of LLN or >150% of ULN if amplitude <80% of LLN in
2 nerves
d. Partial conduction block (>20% amplitude reduction from
distal to proximal stimulation sites if <15% change in
duration between the CMAPs) or abnormal temporal
dispersion (>15% change in duration between distal and
proximal stimulation sites and >20% amplitude reduction
from distal to proximal stimulation sites) in 1 nerve
Definite CIDP: Clinical and EDx parameters, plus
demyelination on nerve biopsy and CSF (for absence of
pleocytosis)
Probable CIDP: clinical and EDx parameters plus CSF analysis
support
Possible CIDP: clinical and EDx parameters
Abbreviations: CMAP, compound motor action potential; EDx, electro-
diagnostic; LLN, lower limit of normal; ULN, upper limit of normal.
Seminars in Neurology Vol. 39 No. 5/2019
criteria, since they do not require nerve biopsy or lumbar
puncture if adequate clinical and EDx criteria are met. Thai-
setthawatkul et al52 incorporated prolonged distal CMAP
duration into the EDx criteria to further promote diagnostic
sensitivity. Magda et al53 proposed a set of diagnostic criteria
based on minimal EDx changes shared by a collection of CIDP
patients. These were adopted in the Neuropathy Association
guidelines for the diagnosis and treatment of CIDP,54 and it was
inclusive of patients with abnormal EDx findings in three
nerves, with findings diagnostic of demyelination in just one
nerve. The more recent European Federation of Neurologic
Societies/Peripheral Nerve Society (EFNS/PNS) criteria55
(►Table 4) expanded on the previous criteria, and also allows
the diagnosis of CIDP with EDx demyelinating findings in one
nerve if additional supportive criteria are met. The EFNS/PNS
criteria have been used in recent and ongoing observational
studies and clinical trials.
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Not all proposed diagnostic criteria are mentioned here.
Attempts to improve the sensitivity of CIDP diagnostic
criteria often decrease their specificity, and vice versa. A
retrospective study comparing CIDP, diabetic neuropathy,
and amyotrophic lateral sclerosis patients showed that the
Magda et al/Neuropathy Association guidelines and the
EFNS/PNS criteria had relatively higher sensitivity (75%
and 73%, respectively), while maintaining relatively high
specificity (83% and 88%, respectively), among the many
proposed criteria.56 Several of the aforementioned criteria
(AAN, Saperstein et al, EFNS/PNS) have parameters for
Table 3 Features of the INCAT diagnostic criteria for CIDP51
Clinical features:
Progressive or relapsing motor and sensory dysfunction of
more than one limb for >2 months.
Reduced or absent reflexes.
EDx parameters for demyelination:
a. Reduced conduction velocity <80% of LLN if
amplitude > 80% of LLN or <70% of LLN if amplitude
<80% of LLN in 2 nerves
b. Prolonged distal motor latency >125% of ULN if amplitude
>80% of LLN or >150% of ULN if amplitude <80% of LLN in
2 nerves
c. Prolonged F-wave latency >120% of ULN if amplitude
>80% of LLN or >150% of ULN if amplitude <80% of LLN in
2 nerves
d. Partial conduction block (>20% amplitude reduction from
distal to proximal stimulation sites if <15% change in
duration between the CMAPs) or abnormal temporal
dispersion (>15% change in duration between distal and
proximal stimulation sites and >20% amplitude reduction
from distal to proximal stimulation sites) in 1 nerve
Diagnosis of CIDP requires one of the following:
• 2 nerves with conduction block or abnormal temporal
dispersion þ 1 other nerve with either abnormal distal
motor latency, conduction velocity or F-wave response. Or
• 3 nerves with either abnormal distal motor latency,
conduction velocity or F-wave response. Or
• If only 2 nerves with significant EDx abnormality, need
unequivocal evidence of demyelination on nerve biopsy.
Abbreviations: CMAP, compound motor action potential; EDx, electro-
diagnostic; LLN, lower limit of normal; ULN, upper limit of normal.

Table 4 Features of the EFNS/PNS diagnostic criteria for CIDP55
Clinical criteria:
Typical: progressive, stepwise, or recurrent symmetric
proximal and distal weakness and sensory dysfunction of all
extremities 2 months and absent or reduced tendon
reflexes, or
Atypical: inclusive of DADS, Lewis-Sumner syndrome,
asymmetrical, pure motor, and pure sensory symptoms
EDx criteria:
Definite: at least one of the following parameters:
(a) Conduction velocity 70% of LLN in 2 nerves
(b) Distal motor latency 150% of ULN (excluding median
neuropathy at the wrist from carpal tunnel syndrome) in
2 nerves
(c) F-wave latency 130% of ULN (150% if CMAP amplitude
<80% of LLN) in 2 nerves
(d) Absent F-wave if distal CMAP amplitude 20% of LLN in 2
nerves, if there is another demyelinating finding in
another nerve
(e) Partial conduction block: 50% amplitude reduction
from distal to proximal stimulation sites if distal CMAP
amplitude 20% of LLN in 2 nerves (if seen in only one
nerve, then requires another demyelinating finding in
another nerve)
(f) Temporal dispersion: >30% duration increase from distal
to proximal stimulation sites in 2 nerves
(g) Distal CMAP duration increased in at least 1 nerve
(median 6.6 milliseconds, ulnar 6.7 milliseconds,
peroneal 7.6 milliseconds, tibial 8.8 milliseconds)
plus one other demyelinating parameter in another
nerve
Probable:
Partial conduction block 30% amplitude reduction
between distal and proximal stimulations sites (excluding
the posterior tibial nerve) if distal negative peak CMAP
amplitude 20% of LLN in 2 nerves. (If seen in only one
nerve, then requires another demyelinating finding in
another nerve.)
Possible:
Only 1 nerve meeting one of the definitive EDx parameters
above
Definite CIDP: Clinical criteria (typical or atypical) plus:
• definite EDx criteria, or
• probable EDx criteria and one supportive criterion, or
• possible EDx criteria and two supportive criteria.
Probable CIDP: clinical criteria (typical or atypical) plus:
• probable EDx criteria, or
• possible EDx criteria and one supportive criterion
Possible CIDP: clinical criteria (typical or atypical) plus
possible EDx criteria
Supportive criteria: 1) CSF protein elevated with <10 WBC.
2) MRI contrast enhancement or hypertrophy of the nerve
roots or plexus. 3)Sensory conduction velocity slowing, or
reduced median (excluding at the wrist) or radial SNAP with
normal sural amplitude, or delayed SSEP. 4) Objective clinical
improvement after immunomodulatory treatment. 5) Nerve
biopsy showing unequivocal evidence of demyelination/re-
myelination.
Abbreviations: CMAP, compound motor action potential; EDx, electro-
diagnostic; LLN, lower limit of normal; ULN, upper limit of normal.
definite, probable, and possible CIDP. While most clinical
trials may require a definitive diagnosis of CIDP, having
parameters for probable and possible CIDP gives clinicians
justification to treat the patient if CIDP is suspected, even if
CIDP Shije and Brannagan 599
they fall short of satisfying all diagnostic parameters for
definite CIDP, considering no one criteria has both perfect
sensitivity and specificity.
Ultimately, the approach to diagnosis is having suspicion
of CIDP when the patient has symmetrical, progressive, or
relapsing weakness for more than two months, involving
distal and proximal extremities, with diminished or absent
reflexes. If there is adequate EDx support for an acquired
demyelinating neuropathy/radiculoneuropathy, with no
other apparent etiology, the diagnosis of CIDP is justified.
If EDx findings are equivocal or if nerve conduction
responses are inadequate to assess for demyelination, other
tests such as CSF analysis, nerve biopsy, and contrast MRI of
the nerve roots or a diagnostic trial of immunotherapy may
offer additional diagnostic value.
Atypical Variants
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The EFNS/PNS criteria has Lewis-Sumner Syndrome, distal
acquired demyelinating symmetric (DADS) neuropathy, and
sensory CIDP included as atypical CIDP.55 Lewis-Sumner
Syndrome, also known as multifocal acquired demyelinating
sensory and motor neuropathy (MADSAM), was initially
described by Lewis and colleagues, who reported chronic
asymmetric sensorimotor demyelinating neuropathy that
resembled mononeuritis multiplex but were diagnosed
with a demyelinating neuropathy by persistent motor con-
duction block in the nerves affected.57 Patients with Lewis-
Sumner Syndrome generally respond to conventional CIDP
treatment, and some of them evolve to a more typical CIDP.58
DADS neuropathy has been used to describe those with only
distal extremity involvement. In this population, nearly two-
thirds may have an IgM kappa monoclonal gammopathy, and
of those, more than half may have anti–myelin-associated
glycoprotein (MAG) antibody.59 DADS neuropathy has been
reported to progress to classic CIDP,60 although it should be
emphasized that only those without IgM gammopathy or
anti-MAG antibodies are more likely to respond to conven-
tional CIDP treatment59,61 and considered an atypical variant
of CIDP.62,63 Pure sensory CIDP has been described, charac-
terized by progressive sensory neuropathy without weak-
ness, although many of these cases also had EDx evidence of
demyelination involving motor nerves. CSF albuminocytolo-
gic dissociation64 and demyelination on nerve biopsy65 may
be seen in these patients as well. It has been reported that
conventional CIDP treatment may stop symptom progression
and improve sensory impairment or sensory ataxia in these
patients.65,66 Some of the pure sensory CIDP patients may
evolve to have motor symptoms eventually, and have a
pattern of classic CIDP with proximal and distal weakness,
although it may be several years from the onset of pure
sensory symptoms before subsequent motor involvement
becomes apparent.66,67 Furthermore, a particular form of
sensory CIDP, named chronic immune sensory polyradiculo-
pathy (CISP), may only involve the preganglionic sensory
nerve roots, with therefore normal sensory nerve action
potentials, and may be demonstrated by abnormal somato-
sensory evoked potential testing or MRI contrast enhance-
ment of posterior nerve roots. These patients may have CSF
Seminars in Neurology Vol. 39 No. 5/2019
600 CIDP Shije and Brannagan
albuminocytologic dissociation as well, and may respond to
conventional CIDP treatment.68
Coexisting Disorders and Mimics
CIDP may coexist with other conditions, and some of these
may cause unrelated neuropathies as well, which may pre-
sent additional diagnostic challenges. Nevertheless, CIDP
patients with these coexisting conditions respond to con-
ventional CIDP treatment. On the other hand, some disorders
may cause neuropathies that mimic or even satisfy the
diagnostic criteria for CIDP but otherwise do not respond
to conventional CIDP treatment. It is important to distin-
guish the conditions that may simply coexist with CIDP from
the mimics.
CIDP and diabetic (type 1 and type 2) neuropathy may
have overlapping clinical and EDx features. The question of
whether diabetes increases the risk of CIDP has been
debated.20,69,70 Nevertheless, diabetic patients with CIDP
respond to conventional CIDP treatment,71,72 although care-
ful consideration of treatment options should be given to
avoid worsening the underlying diabetes (i.e., caution with
corticosteroids).
Various forms of neuropathy can occur in different stages
of human immunodeficiency virus (HIV) infection, and CIDP
has been reported in HIV-positive patients as well.73,74 It is
important to note that HIV patients with CIDP may have CSF
pleocytosis (often with CSF WBC >10/mm3).75 In addition to
treating the underlying HIV infection, the coexisting CIDP
often responds to conventional CIDP treatment as well.73,76
Monoclonal gammopathy of undetermined significance
(MGUS), including immunoglobulin-A (IgA), immunoglobu-
lin-G (IgG) and immunoglobulin-M (IgM) gammopathy, may
coexist or even cause neuropathy. When CIDP patients have
coexisting MGUS, they may respond to conventional treat-
ment similar to CIDP patients without MGUS.77 However,
there is a number of IgM class autoantibodies, including anti-
MAG, GD1b, and sulfatide antibodies, which can be asso-
ciated with neuropathies with demyelinating features. Anti-
MAG antibody is associated with a distal sensorimotor
neuropathy with prominent distal demyelination, and is
treated as a separate disorder, often responding better to
rituximab and not intravenous immunoglobulin (IVIG).78–81
Anti-GD1b antibodies can be associated with chronic-ataxic-
neuropathy-ophthalmoplegia-m-protein-agglutination-dis-
ialosyl-antibodies (CANOMAD) syndrome, and is often
responsive to immunotherapy.82,83 Antisulfatide antibodies
may be associated with a predominantly sensory or distal
sensorimotor neuropathy.84 When a monoclonal protein is
present, it is especially important to rule out a plasma cell
dyscrasia (i.e., multiple myeloma), and a skeletal survey is
recommended to evaluate for osteosclerotic myeloma. Par-
ticularly, lambda-restricted monoclonal gammopathy may
be associated with polyneuropathy, organomegaly, endocri-
nopathy, M-protein, and skin changes (POEMS) syndrome
(i.e., hyperpigmentation and hemangioma). An elevated vas-
cular endothelial growth factor (VEGF) level can be a diag-
nostic marker for POEMS syndrome and will also decrease
with successful treatment. Neuropathy in POEMS syndrome
Seminars in Neurology Vol. 39 No. 5/2019
often has demyelinating features similar to CIDP,85,86 but
typically does not respond to conventional CIDP treatment.
Charcot-Marie-Tooth type 1 (CMT1), being a hereditary
demyelinating neuropathy, can have similar clinical and
electrodiagnostic features to CIDP. It has been entertained
whether CMT1A, being associated with a PMP22 mutation,
increases the risk of developing CIDP, considering anti-
PMP22 antibodies have been isolated in the serum of CIDP
patients.41 It should be noted that most patients with CMT1
do not respond to immunotherapy; however, coexisting
inflammatory neuropathy has been reported in CMT type
1A, 1B, and CMT X.87–89 The typical clinical course of CMT1 is
that of a gradually progressive length-dependent motor and
sensory polyneuropathy. However, if the patient has super-
imposed acute/subacute, relapsing, or stepwise deteriora-
tion, in a non–length-dependent manner, a suspicion of
superimposed CIDP may be raised. CIDP and CMT1 may
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have similar EDx features of demyelination; however, multi-
focal slowing and conduction block are more likely to be seen
in CIDP rather than CMT1.90 These and additional associated
conditions, and mimics, are listed in ►Table 5.
Treatment and Outcome
Corticosteroids
One of the hallmarks of CIDP is that it responds to corticos-
teroid treatment.1,2 An early randomized controlled trial
demonstrated that patients who received prednisone at an
equivalent of 60 mg a day, tapered over three months,
resulted in a better neuropathy disability score (NDS), later
renamed neuropathy impairment score (NIS), compared
with placebo.100 Adverse effects of long-term high dose
steroid use include dyspepsia, hyperglycemia, osteoporosis,
weight gain, Cushingoid features, susceptibility for infection,
and mood and sleep disturbance. Subsequently, various
investigators reported that pulsed steroid regimens are
also effective, while causing relatively less side effects com-
pared with daily oral steroids.101,102 The efficacy of pulsed
oral steroids was demonstrated in the PREDICT study,103
where pulsed oral dexamethasone 40 mg a day for 4 days,
every 4 weeks, showed similar efficacy but less adverse
effects (i.e., Cushingoid features and sleeplessness) com-
pared with daily prednisolone 60 mg tapered over 6 months
Table 5 Associated conditions and mimics
Conditions in which coexisting CIDP may be seen.
Diabetes, HIV, monoclonal gammopathies, Charcot-Marie-
Tooth type 1, other autoimmune disorders (i.e., lupus,
inflammatory bowel disease, membranous
nephropathy),91–93 chronic hepatitis,4 post-organ
transplant,94 malignancy,95 TNF-α inhibitor use (not known
to cause neuropathy otherwise).96 Immune checkpoint
inhibitor use97,98
Other neuropathies that may mimic CIDP clinically and
electrodiagnostically
Anti-MAG neuropathy, multifocal motor neuropathy,
neuropathy in POEMS, hereditary transthyretin amyloid
neuropathy.86,99

for CIDP patients. Subsequently, another randomized con-
trolled trial comparing IV methylprednisolone 0.5 g a day for
four consecutive days/month and IVIG 0.5 g/kg a day for four
consecutive days/month over a 6-month period, demon-
strated that in the patients who responded to treatment,
the degree of improvement was similar between the two
groups.104 The IV methylprednisolone group had more
patients drop out of the study, mostly citing lack of improve-
ment as the reason, compared with the IVIG group. However,
it should be noted that the previous PREDICT study had
shown the median time to improvement from pulsed oral
steroid was 17 weeks (ranging from 13–20 weeks). There-
fore, the likelihood of improvement from IV methylpredni-
solone could have been underestimated due to premature
treatment discontinuation. Long-term follow-up analysis of
the same study showed that ultimately both groups had a
similar percentage of relapse after treatment discontinua-
tion, although the IV methylprednisolone group had a sig-
nificantly longer median time to relapse (median:
14 months, range: 1–31 months) compared with the IVIG
group (median: 4.5 months, range: 1–24 months).
Immunoglobulin
Randomized controlled studies have demonstrated that CIDP
patients who received an initial treatment of IVIG could have
improvement of their strength and disability scores within 2
to 4 weeks of the infusion.51,105 It was also shown that
relapsing symptoms may occur from 3 to 22 weeks after
an initial treatment of IVIG at 2 g/kg, and additional IVIG can
be used to maintain the benefit of the initial treatment.106
Subsequently, it was demonstrated in the ICE study,107
starting IVIG with a 2 g/kg loading dose, followed by a
maintenance regimen of 1 g/kg every 3 weeks, can maintain
the improvement in their disability score at 24 weeks, and up
to 48 weeks in the extended phase of the study. Nevertheless,
patients may still report relapses sooner than 3 weeks after
infusion. Maintenance IVIG as frequently as every two weeks
has been reported to be effective and well tolerated in some
cases.108
Immediate adverse reactions following IVIG administra-
tion are often mild, including transient flu-like symptoms,
headache, flushing of the face, change in blood pressure, and
tachycardia. Minor reactions can often be ameliorated by
pretreatment with analgesics, antihistamines, and corticos-
teroids, and by slowing the infusion rate. More serious
adverse effects may include thrombosis, renal injury, aseptic
meningitis, neutropenia, and pseudohyponatremia. Anaphy-
laxis is rare but its risk is increased in those with IgA
deficiency. Avoiding sucrose-containing preparations may
decrease the risk of renal injury. Decreasing the infusion rate
(i.e., at least over 8 hours or no greater than 3–5 g/hr) may
decrease the risk of thrombosis, renal failure, and aseptic
meningitis.109,110 There is no evidence of any particular
brand or preparation of IVIG being more efficacious or having
more adverse effects.111,112 However, there are anecdotes of
patients tolerating one brand of IVIG better than another.
For patients who respond to IVIG but prefer more auton-
omy, subcutaneous immunoglobulin-G (SCIG) may be a
CIDP Shije and Brannagan 601
reasonable alternative. It was shown that SCIG of 0.4 g/kg
(over 2–3 infusions) weekly for 5 weeks had similar efficacy
as IVIG 2 g/kg over 5 days as initial treatment in improving
muscle strength at 10 weeks, although peak improvement in
patients receiving IVIG occurred sooner with IVIG than in
those receiving SCIG. Therefore, severely disabled patients
may benefit more from IVIG initially, and SCIG may be
considered as maintenance treatment.113 The efficacy of
SCIG as a maintenance treatment in patients who previously
responded to IVIG has been demonstrated as well.114,115
SCIG at a maintenance dose of 0.2 to 0.4 g/kg weekly may
be used.116 SCIG had less moderate and/or systemic adverse
effects compared with IVIG based on a meta-analysis,
although a mild transient injection site reaction may be
more common with SCIG.117
Plasmapheresis
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Plasmapheresis is effective as well, its efficacy as initial
treatment is comparable to IVIG in a 6-week study.118
Usually, 1 to 1.5 times body volume is exchanged at each
session, and 5 to 10 exchanges (2 to 3 exchanges a week as
tolerated) may be considered for an initial course of treat-
ment. Plasmapheresis may be used as a first line initial
therapy, especially in those with severe symptoms, consider-
ing that an improvement is often seen within the first week
or after a couple of exchanges. However, more than half of
patients have relapsing symptoms within 1 to 2 weeks after
stopping plasmapheresis.119 Its adverse effects may include
hemodynamic disturbances, depletion of coagulation fac-
tors, and venous access-related complications. Therefore, it
may be less ideal as a maintenance therapy option compared
with corticosteroids and IVIG.
Variability in Treatment Response and Other
Therapy Options
Steroids, IVIG, and plasma exchange remain the most evi-
dence-based, first-line treatment options for CIDP,120–122
and their dosing strategies are summarized in ►Table 6.
One retrospective study showed that of the CIDP patients
(about one-third) who did not respond to an initial first-line
treatment, about half may respond to treatment after
switching from steroids to IVIG or plasmapheresis, or after
switching from IVIG or plasmapheresis to corticosteroids.
Still, there remained 19% of patients who did not response to
either steroids, IVIG, or plasmapheresis.123 Considering
about one-fifth of CIDP patients may not respond to steroids,
IVIG, or plasmapheresis, there is a need for other treatment
options. Various chemotherapy and other immunomodula-
tory agents, that is, azathioprine,124 mycophenolate mofe-
til,108,125 methotrexate,126 cyclophosphamide127(with or
without fludarabine108), cyclosporine,128,129 interferon
(IFN) α,130,131 IFN β-1a,132 and rituximab,133,134 have been
reported to offer some benefit in refractory cases. However,
these treatments lack support from large clinical trials.
Randomized controlled studies with azathioprine,135 IFN
β-1a,136,137 methotrexate,138 and Fingolimod139 failed to
show significant efficacy for CIDP. It should be noted that
the azathioprine study only evaluated its efficacy as an
Seminars in Neurology Vol. 39 No. 5/2019
602 CIDP Shije and Brannagan

Table 6 First-line treatment options

Rx Dosing options Precautions and considerations with Rx

Corticosteroids Prednisone 60 mg daily for one month, then taper
gradually over 3–6 months.
Dexamethasone 40 mg/day  4 days every
four weeks for 6 months, then taper gradually if
able
IV methylprednisolone 0.5 g/day  4 days
very month for 6 months, then taper gradually if
able
Immunoglobulin IVIG 2 g/kg over 4–5 days initially, then 1 g/kg over
1–2 days every three weeks as maintenance (may
increase frequency to every 2 weeks in some cases)
SCIG, initial 0.4 g/kg/wk (over 2–3 infusion).
Maintenance 0.2 to 0.4 g/kg weekly.
Plasmapheresis 1 to 1.5 times total body volume per exchange, for
total of 5 to 10 exchanges (2–3 exchanges/week as
tolerated) as initial treatment.
Recommend GI prophylaxis, glycemic and BP
monitoring, Ca & Vit-D supplement.
Improvement may not be seen for 3 to 4 months
IgA deficiency increases risk of anaphylaxis.
Caution for renal insufficiency. Vigilance for DVT,
neutropenia, hemodynamic instability. Avoid high
rate of infusion. Can pretreat with Benadryl,
Tylenol, and steroid for mild immediate reactions
Monitor for hemodynamic disturbance and
complications associated with venous access, i.e.,
thrombosis, thrombophlebitis, line infection/

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Schedule for maintenance exchanges may vary and sepsis, etc.
depend on relapsing symptoms

Table 7 Other immunomodulatory/chemotherapy agents

adjunctive therapy to prednisone, and the duration of the
study might have been suboptimal.135 A Cochrane review of
some of these studies concluded that azathioprine, metho-
trexate, and IFN β-1a offer no significant benefit, although a
small or moderate benefit could not be ruled out.140
One retrospective multicenter observational study in Italy
showed that of the 110 nonresponders to conventional
treatment (i.e., steroids, IVIG, and plasmapheresis), 37%
had a favorable response from other immunosuppressive/
immunomodulatory treatments including azathioprine,
cyclophosphamide, methotrexate, cyclosporine, rituximab,
and IFN-α, with an average improvement of 1.9 points on the
Rankin Scale.124 There was no significant difference in the
treatment responses between these agents. It was also noted
that azathioprine was utilized the most, presumably due to
its ease of use; cyclophosphamide had the highest percent of
treatment responders, methotrexate had the lowest percent
of side effects, and cyclosporine had the highest percent of
discontinuation due to side effects. It should be noted that
some of these agents, such as azathioprine and mycopheno-
late, may not show benefit for many months after the start of
treatment. There is no standardized dosing for these treat-
ments, and dosing strategies based on the aforementioned
case reports and retrospective study are illustrated in
►Table 7. It is advised that one should be familiar with the
potential adverse effects of these immunomodulatory/che-
motherapy agents prior to use, and collaboration with rheu-
matology or oncology may be considered.
Some additional immunomodulatory treatments have
been reported to be effective in case reports and small
cohorts, but may not be ideal options for various reasons.
Alemtuzumab has been reported to decrease the IVIG
requirement in IVIG-dependent CIDP patients in a small
cohort study.141 However, its risk of causing other serious
autoimmune disorders may not justify its use when the
patient is otherwise well controlled on maintenance IVIG.
Tacrolimus has been reported to be beneficial in a case
reported to be beneficial for CIDP refractory to first-line
treatments, in case reports and retrospective observations
Azathioprine 100–200 mg/day
Cyclophosphamide 1 g/m2 IV/month, or 2 mg/kg oral/
day. High dose: 200 mg/kg IV over
4 days. (or 250 mg/m2/day with
fludarabine 25 mg/m2/day for 3 days
every 5–6 days)
Mycophenolate 500–3000 mg/day (typically
mofetil 2000 mg/day), divided twice a day
Rituximab 375 mg/m2/week  4 weeks
Methotrexate 7.5–15 mg/week
Cyclosporin A 100–300 mg/day or 5 mg/kg/day
(keeping plasma concentration
between 100 and 150 ng/mL)
report142 however, it also has been described to be asso-
ciated with demyelinating neuropathy.143 Etanercept, a
tumor necrosis factor (TNF)-α inhibitor, has been reported
to be effective in some CIDP patients refractory to or intol-
erant of first-line treatments144; however, TNF-α inhibitor is
also associated with developing CIDP.96
Finally, stem cell transplantation may be considered in
select cases after carefully weighing its potential risks and
benefit. There is a report of allogenic hematopoietic stem cell
transplant in a CIDP patient who achieved full recovery
within 8 months and had no relapse at 6.5 year follow
up.154 There are also reports of autologous stem cell trans-
plantation leading to disease remission145–147 however,
relapsing disease was also reported in two of those cases
at 2 and 5 years post transplant. In a retrospective review of
11 patients who received autologous hematopoietic stem
cell transplant for refractory CIDP, 3 patients had relapsing
disease (1 at 14 months and 2 at 23 months), although 8 of
those 11 patients were able to maintain drug-free remission

Seminars in Neurology Vol. 39 No. 5/2019


at a median follow up time of 28 months.148 One of the
refractory patients achieved remission again after a second
autologous stem cell transplantation. The authors reported
short-term complications that included CMV and EBV reac-
tivation, and pancreatitis. A phase 2 open-label clinical trial
for nonmyeloablative autologous hematopoietic stem cell
transplantation in CIDP patients is ongoing.
Paranodal Antibodies and Response to Treatment
It is important to be aware that a small percentage of CIDP
patients have IgG class 4 (IgG4) autoantibodies to paranodal
proteins, that is, neurofascin-155 and contactin-1.44 Anti–
neurofascin-155 antibody is associated with higher inci-
dence of ataxia and tremor, younger age (20–30 s) of
onset,6,8,149 and higher CSF protein levels,9 and a small
percentage of them may have concomitant demyelinating
disease involving the central nervous system.7 Anti–contac-
tin-1 is also associated with a higher incidence of ataxia and
tremor,8,11 and it may have a more rapidly progressive
onset.10,150 CIDP patients with anti–neurofascin-155 and
contactin-1 antibodies have poorer response to IVIG in
general, although they may have more a favorable response
to corticosteroids.6–9,11,149 In addition, rituximab has been
reported to be effective in CIDP patients with neurofascin-
155 and contactin-1 auto-antibodies, including those who
have failed both IVIG and corticosteroids.10,149,151,152
Outcome
Though over 50% of patients with CIDP are disabled at some
time during the disease,21 the majority of CIDP patients can
remain stable with ongoing treatment. A CIDP Disease
Activity Status (CDAS) classification (►Table 8) was devised
by a panel of experts, based on outcome data from long-
term follow up of 106 CIDP patients.153 Remission, defined
as being stable off treatment for less than 5 years, was seen
in 20% of patients. Furthermore, 11% were considered
cured, if clinically stable for at least 5 years off treatment.
Therefore, patients with stable disease on treatment for at
least 1 year can be considered for tapering of their ther-
apeutic regimen. There is no standardized approach to
tapering treatments. Gradually decreasing the therapy dos-
ing and frequency, with close monitoring for relapsing
symptoms, is advised.
Conclusion
Our collective understanding of CIDP has evolved over time.
Typical cases and atypical variants, as well as other mimics,
can be recognized with a thoughtful approach to diagnosis.
Corticosteroids, IVIG, and plasmapheresis have the best
evidence as first-line treatments, while steroids and IVIG
are often preferred as maintenance therapy. However, if a
favorable response is not seen with one of these first-line
treatments, switching or escalating treatment, including
utilizing other immunomodulatory/chemotherapy, may
offer benefit. CIDP appears to be a heterogeneous group of
disorders. While ample evidence points to a prominent cell-
mediated autoimmune process in most cases, a humoral-
CIDP Shije and Brannagan 603
Table 8 CIDP Disease Activity Status (CDAS)153
1. Cure: off treatment 5 years
A. Normal exam
B. Abnormal exam but at least stable
2. Remission: off treatment <5 years
A. Normal exam
B. Abnormal exam but at least stable
3. Stable active disease: on treatment at least 1 year
A. Normal exam
B. Abnormal exam but at least stable
4. Improvement: on treatment between 3 months and 1 year
A. Normal exam
B. Abnormal exam but at least stable
5. Unstable active disease: abnormal exam with progressive
or relapsing course
A. Treatment naïve or treatment < 3months
B. Off treatment
C. On treatment
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mediated (antibody-driven) autoimmune process may play a
role in some cases. Perhaps, different CIDP patients may have
different pathophysiologies that influence their response to
various treatments. This is evidence from the recent discov-
ery that CIDP patients who have IgG4 class antibodies to
paranodal proteins, such as neurofascin-155 and contactin-
1, have certain distinct clinical features and respond poorly
to IVIG but may have a better response to corticosteroids or B
cell depleting agents such as rituximab. As our knowledge of
CIDP continues to evolve, it may be possible to tailor its
treatment based on the immunopathological characteristics
of individual patients and improve their overall outcome.
Conflict of Interest
Dr. Brannagan has received compensation for consulting
from CSL-Behring, Grifols and Shire.
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