Diagnóstico CIDP
Diagnóstico CIDP
Diagnóstico CIDP
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Received: 1 July 2022 Revised: 12 August 2022 Accepted: 16 August 2022
DOI: 10.1002/mus.27708
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1
Department of Neurology, University of
Minnesota, Minneapolis, Minnesota Abstract
2
Department of Neurology, Cedars-Sinai Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic immune-mediated
Medical Center, Los Angeles, California
peripheral form of polyneuropathy. No reliable diagnostic biomarkers are available by
Correspondence which to make the diagnosis of CIDP. As a result, diagnosis of the condition can be chal-
Jeffrey A. Allen, Department of Neurology,
University of Minnesota MMC 295, 420 lenging. Many patients are not recognized early in the disease course, and on the other end
Delaware Street SE, Minneapolis, MN 55455. of the spectrum both establishing early and accurate diagnosis as well as avoiding misdiag-
Email: [email protected]
nosis and overtreatment. Identification of the hallmark clinical, electrophysiological, and
Funding information laboratory features of the disease are critical to facilitate rapid diagnosis, while an under-
European Academy of Neurology
standing of diagnostic pitfalls can help prevent misdiagnosis. Since the original description
of CIDP in the 1970s, over 15 sets of diagnostic criteria have been proposed. The criteria
published in 2021 by the European Academy of Neurology / Peripheral Nerve Society
(EAN/PNS) were developed for use during routine clinical care and are available in the pub-
lic domain. These criteria provide clinicians with an invaluable resource by which the data
collected during the evaluation of the patient with possible CIDP can be interpreted. One
point of importance that bridges diagnosis to treatment is objectification of the treatment
response. Interpretation of how patients respond to treatment drives both long-term treat-
ment paradigms and the diagnosis at which these treatments are aimed. Although no
approach is perfect, utilization of strength impairment and disability outcomes in clinical
practice can help unravel the difficulties in interpreting response to treatment. Just as
improvement in these outcomes is considered diagnostically supportive, the absence of
objective benefit argues against it and should prompt reconsideration of a CIDP diagnosis.
KEYWORDS
Autoimmune, CIDP, Diagnosis, Guidelines, Misdiagnosis, Neuropathy
Muscle & Nerve. 2022;66:545–551. wileyonlinelibrary.com/journal/mus © 2022 Wiley Periodicals LLC. 545
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546 ALLEN AND LEWIS
electrophysiologically by abnormalities on nerve conduction studies “typical” CIDP have relatively symmetric proximal and distal motor
that reflect demyelination of peripheral nerve. and sensory symptoms, the heterogeneous clinical features of the dis-
Three important observations should be considered when ease have been well described. Recognized variants that still fall under
approaching CIDP treatment. First, it is treatable. Between 80% and the CIDP syndromic umbrella include distal CIDP (sometimes referred
90% of patients benefit from one of the first-line CIDP immunother- to as distal acquired demyelinating symmetric [DADS] type), multifo-
apies.4 Second, disability may be substantial. At some point during cal CIDP (also called Lewis-Sumner syndrome, or multifocal acquired
their illness, more than half of CIDP patients are unable to live inde- demyelinating sensory and motor [MADSAM]), sensory CIDP, and
pendently.1,4 Third, diagnostic delay is common. It has been demon- motor CIDP. Although CIDP variants differ with respect to the modal-
strated that the average time between symptom onset and diagnosis ity that predominates or the part of the body that is preferentially
ranges between 12 and 40 months.5,6 The delay in diagnosis leads to affected, all variants have in common weakness and/or numbness as
accumulation of axonal injury, which in turn increases disability that the defining clinical features.
may be irreversible despite treatment. These observations have rightly Since the disease was described in the 1970s, more than 15 sets
placed a premium on rapid diagnosis and early initiation of treatment of CIDP diagnostic criteria have been developed, a fact that points to
before irreversible disability develops. the difficulties in defining a disorder without a reliable diagnostic bio-
Consider next the frequency with which CIDP is misdiagnosed logic marker. Although many early criteria were developed with clini-
and overtreated. In one series of patients treated at a tertiary care cal trials in mind, modern criteria are meant to be used during routine
hospital, 47% of those with a referral diagnosis of CIDP did not clinical care. Over the last decade, the European Federation of Neuro-
have the condition.6 Misdiagnosis rates in community-based studies logic Society / Peripheral Nerve Society (EFNS/PNS) diagnostic cri-
6–8
are even higher. Many patients carrying an incorrect diagnosis of teria have been the most widely used guideline in clinical practice and
CIDP are nonetheless treated for long periods with costly and in clinical trials.12 These guidelines, first published in 2006 and
potentially harmful immunotherapies. What about patients with true updated in 2010, have been shown to have a sensitivity of 73% to
disease? Despite the chronic “C” attached to the name of the ill- 91% and a specificity of 66% to 88%.13 In 2021, the criteria under-
ness, CIDP does not always require long-term immunotherapy. went a second revision, and now are called the European Academy of
Although the cure rate may be low, about 30% of patients with Neurology and Peripheral Nerve Society (EAN/PNS) criteria.13 The
CIDP achieve a durable period of drug-free remission, and may not updated criteria more directly recognize that, although the diagnosis
need long-term immunotherapy (although many are treated none- of typical CIDP with symmetric proximal and distal weakness is not
9
theless). CIDP prescribing practices vary widely, often deviating overly challenging, the variants can be tricky and are difficult to define
from treatment guidelines, and are rarely driven by collection of by a simple or standard diagnostic guideline. EAN/PNS 2021 criteria
objective measures of treatment response to justify ongoing immu- highlight unique features, which should be explored in patients sus-
notherapy exposure.10 pected to have one of the CIDP variants, and they provide useful
CIDP is a disease without a diagnostic biomarker. Balancing rapid advice on alternative diagnostic considerations specific to each of the
diagnosis and early treatment initiation with accurate diagnosis and variants.
avoidance of overtreatment is not easy. In this review we focus on Nerve conduction studies (NCS) play an instrumental role in the
the diagnostic pearls that can be leaned on to improve expeditious diagnosis of CIDP. Electrophysiological findings that support periph-
diagnosis and avoid misdiagnosis. eral nerve demyelination include the presence of motor distal
latency prolongation, slowed motor conduction velocity, motor con-
duction block, temporal dispersion, and prolonged or absent F
2 | D I A G NO S I N G C I D P : KE Y S T O M A K I NG waves. Sensory responses are often absent or attenuated in the
A RAPID AND SUCCESSFUL DIAGNOSIS upper and lower limbs. When abnormalities on NCS are uncovered
in clinical practice it can be challenging to identify whether any
Throughout the 20th century the cardinal clinical, electrodiagnostic, given “demyelinating” finding is abnormal to the extent that it
histopathological, and laboratory features of what we now call CIDP unequivocally supports peripheral nerve demyelination. We have
were described, culminating in the naming of the disorder by Peter found electrodiagnostic guidelines to be an invaluable resource
Dyck in 1975.11 Although much has been learned about CIDP since it when working through ambiguous electrophysiological findings dur-
was named, the processes of how a diagnosis is reached are largely ing routine clinical care (Table 1).12,14 The absence of electrophysio-
unchanged. CIDP remains a clinical and electrophysiological diagnosis logical evidence of demyelination should prompt exploration for an
that can be supported by laboratory, imaging, and treatment data. alternative diagnosis.
The challenge is in how to integrate these features accurately and In the event that the clinical and electrophysiological components
rapidly. of the disease are well-defined, a diagnosis of CIDP can confidently
At its core, CIDP is a clinical diagnosis. The disease is defined by be made provided there is no better explanation. Consideration of
numbness and weakness that evolves over 2 months or more in a pro- alternative explanations is important in all patients with suspected
gressive or relapsing pattern, with muscle stretch reflexes invariably CIDP, especially when the clinical or electrophysiological data are
reduced or absent on examination.12,13 Although patients with equivocal or if there are unique circumstances that pertain to a
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ALLEN AND LEWIS 547
Note: Adopted from European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and management of chronic inflammatory
demyelinating polyradiculoneuropathy: Report of a joint task force---second revision.14
particular patient. In cases where the diagnosis is uncertain, the collec- immunologically active.15 Pain typically in the distal extremities may
tion of supportive data can be helpful to increase diagnostic confi- affect one third or more of patients.16 In rare cases, tremor (50% of
dence. Diagnostic data considered supportive of CIDP includes patients),17 mild autonomic dysfunction (25%),18 and cranial nerve
cerebrospinal fluid (CSF) albuminocytological dissociation, characteris- dysfunction (5% to 20%),19 usually involving the facial nerve, will
tic imaging findings on magnetic resonance imaging (MRI) or ultra- develop. From a management perspective, these symptoms should
sound, or histopathological hallmarks on nerve biopsy. We have also not be dismissed, but from a diagnostic perspective caution is needed
found testing for nodal and paranodal antibodies as well as screening because an overreliance on any of these features introduces a possi-
for genetically determined neuropathies of high yield in selected clini- ble misdiagnosis. Although pain, fatigue, or similar nonspecific symp-
cal settings. Ancillary testing data are presented in Table 2, with a toms may be part of CIDP, if the condition is defined by those
focus on when collection of the data is helpful and when it is not symptoms absent the hallmarks of numbness and/or weakness in a
helpful. pattern and distribution that conforms to one of the defined variants,
then the diagnosis of CIDP is unlikely to be correct.
The diagnostic challenges unique to the CIDP variants are worth
3 | T H E M I S DI A G N O S I S OF CI DP : S I RE N S highlighting. Relatively speaking, “typical” CIDP is a straightforward
AND PITFALLS diagnosis when patients manifest a neuropathy that evolves over
2 months or more and is characterized by symmetric proximal and dis-
At each step in the diagnostic process, sirens threaten to lure the diag- tal weakness. Although even in this setting electrophysiological stud-
nostician astray and pitfalls decorate the landscape. Recognizing these ies are important to provide evidence of peripheral nerve
obstacles provides insight on how they can be avoided. As noted pre- demyelination, a diagnosis of “typical” CIDP can confidently be made
viously, CIDP is a syndrome with a “typical” phenotype and variants.14 provided there is no serum paraprotein, suspicion for a genetic abnor-
Although the motor and sensory symptoms are the defining clinical mality, or other apparent explanation for the symptoms.20 Unlike typi-
CIDP features in all forms of CIDP, other symptoms and signs may cal CIDP, the variants can be more diagnostically challenging. Distal
also be present. Fatigue is a common symptom in patients during all CIDP is easy to confuse with more common length-dependent axonal
phases of the illness, even when the disease is no longer neuropathies or genetically determined neuropathies. Multifocal CIDP
548
Data Classic finding in CIDP When is it helpful When is it not helpful Caution
CSF Protein ", WBC normal • CIDP clinically suspected but only • If diagnostic criteria are already met • Not diagnostic of CIDP if clinical and
meet “possible” criteria • Mild or moderate elevations in the NCS criteria are not fulfilled
• If infectious or malignant etiology context of diabetes, age >50 years, or • CSF protein may be " in diabetes and
(rather than CIDP) possible or advanced spondylosis other neuropathies (eg, CMT, hTTR)
suspected • Normal protein increases with age.
Rigorous age cut-offs not established
MRI Nerve or nerve root enlargement or • CIDP clinically suspected but only • If diagnostic criteria are already met • Not diagnostic of CIDP if clinical and
enhancement meet “possible” criteria • If institutional experience and NCS criteria are not fulfilled
Ultrasound Nerve or plexus enlargement • If spinal cord pathology is suspected expertise in nerve imaging is • Does not distinguish CIDP from other
• In multifocal CIDP if focal nerve unavailable immune (eg, MMN) or genetic (eg,
compression by mass is possible or CMT) neuropathies
suspected • Requires skilled interpreter (MRI and
ultrasound)
• Costly (MRI)
• Normative data lacking (MRI)
Nerve biopsy Thinly myelinated axons, onion bulbs, • CIDP is suspected but cannot be • Clinically mild or moderate cases • Diagnostic yield is low
perivascular macrophage collections confirmed with clinical, laboratory, • If surgical and pathological experience • May result in morbidity
imaging, and electrodiagnostic studies and expertise is unavailable
• If amyloidosis, sarcoidosis, nerve
sheath tumors or neurofibromatosis
possible or suspected
Monoclonal Normal (no monoclonal protein) • All patients with suspected CIDP • Not all monoclonal proteins are • If IgA and IgG paraprotein, especially
gammopathy • Testing should include SPEP, serum relevant. If plasma cell malignancy or with a lambda light chain, consider AL
testing and urine immunofixation proliferative disorder excluded then amyloid, POEMS, cryoglobulinemic
classified as MGUS vasculitis, lymphomatosis
• If CIDP with MGUS then CIDP • If IgM, consider anti-MAG testing
treatment proceeds as if • If there is any monoclonal protein,
without MGUS consider hematology referral,
especially if paraprotein is high or
rising level or if B-symptoms.
Antibody testing Usually none • Nodal/paranodal antibodies (anti- • Typical CIDP with unequivocal Presence of a nodal or paranodal antibody
NF155, anti-NF140/186, anti-CNTN1, treatment response or anti-MAG antibody strongly implies
anti-Caspr1) if tremor, ataxia, or IVIg • If laboratory for antibody analysis a different diagnosis and treatment
treatment failure meeting quality standards is not approach than CIDP
• Anti-MAG if IgM paraprotein or in available
distal CIDP
ALLEN AND LEWIS
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ALLEN AND LEWIS 549
Abbreviations: CIDP, chronic inflammatory demyelinating polyneuropathy; CSF, cerebrospinal fluid; CMT, Charcot-Marie-Tooth disease; CNTN1, contactin 1; IVIg, intravenous immunoglobulin; hTTR, hereditary
genetically determined, or traumatic causes. Motor CIDP mimics
neuropathy; NCS, nerve conduction studies; NF, neurofascin; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein skin changes; SPEP, serum protein electrophoresis; WBC, white
Variants of undetermined significance include multifocal motor neuropathy and motor neuron disorders,
transthyretin; IgM, immunoglobulin M; MRI, magnetic resonance imaging; MGUS, monoclonal gammopathy of undetermined significance; MAG, myelin-associated glycoprotein; MMN, multifocal motor
whereas sensory CIDP can be confused with a host of neuropathic
and non-neuropathic conditions that have a disturbance of skin sensa-
tion. The EAN/PNS CIDP diagnostic guideline14 thoughtfully provides
a list of alternative diagnostic considerations for typical CIDP and
may be detected
less than 30 C or upper limb of less than 33 C may result in errone-
ous distal latency prolongation or conduction velocity slowing.
history of neuropathy
Amplitude and negative peak area reductions are also prone to mis-
• Distal CIDP variant
blood cell.
(such as prominent distal atrophy and pes cavus), which raise suspi-
cion for a genetically determined neuropathy. When these features
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550 ALLEN AND LEWIS
are present, or if there are other reasons that the diagnosis is less cer-
Visual Analogue Scale; INCAT, inflammatory neuropathy cause and treatment; I-RODS, Inflammatory Rasch-built Overall Disability Scale; mISS, modified Inflammatory Neuropathy Cause and Treatment sensory
Abbreviations: CAP-PRI, Chronic Acquired Polyneuropathy Patient-Reported Index; CIDP, chronic inflammatory demyelinating polyneuropathy; EQ-5D, EuroQoL 5-Dimension Questionnaire; EQ-VAS, EuroQoL
tain, supportive testing may be diagnostically helpful, but caution
should be exercised. CSF, imaging, nerve biopsy findings, and
≥2 points
4 | M O N I T O RI N G T R E A T M E N T RE S P O N S E :
≥1 point
Free
Free
Free
Free
Free
Free
Physician
Physician
scale; TUG, timed up and go; PGIC, patient global impression of change.
3-5 min
3-5 min
<1 min
<5 min
<1 min
<5 min
<1 min
value (Table 3). Although it may appear daunting to add another ele-
ment to the physical examination during a busy clinic, most outcomes
can be collected quickly at minimal to no cost. The data gained are
MMT with MRC
and, in turn, what that says about the patient's diagnosis or long-term
CAP-PRI
EQ-VAS
I-RODS
EQ-5D
INCAT
treatment approach.
PGIC
mISS
TUG
Gait impairment
5 | CONC LU SIONS
Quality of life
Assessment
Sensation
Disability
TABLE 3
Strength
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R.A.L. has served as a consultant for Argenx, Biotest, CSL Behring,
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Grifols, Momenta (J&J), Sanofi Genzyme, Pfizer, and UCB; has
of Neurological Societies/Peripheral Nerve Society guideline on man-
received speaker honoraria and served on advisory boards for Akcea, agement of chronic inflammatory demyelinating polyradiculoneuropa-
Alnylam, CSL Behring, Grifols, and Medscape; and has received royal- thy: report of a joint task force of the European Federation of
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DATA AVAI LAB ILITY S TATEMENT
Academy of Neurology/Peripheral Nerve Society guideline on diag-
Data sharing not applicable to this article as no datasets were gener- nosis and management of chronic inflammatory demyelinating polyra-
ated or analysed during the current study" cd_value_code="text diculoneuropathy: report of a joint task force---second revision. J
Peripher Nerv Syst. 2021;26:242-268.
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