Enhancing the Patient Journey in CIDP: Strategies for Improved Dete...

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Enhancing the Patient Journey in CIDP:
Strategies for Improved Detection and
Individualized Management
Karissa Gable, MD
P...

Disclosures
Advisory board or panel: Argenx
Consultant: Argenx, CSL Behring, Dianthus, Grifols, Immunovant,
InCircle, and ...

Learning Objectives
CIDP = chronic inflammatory demyelinating polyneuropathy.
Describe signs, symptoms, and assessments th...

CIDP: Epidemiology,
Clinical Burden, and
Unmet Treatment Needs

Epidemiology of CIDP
Incidence: 0.2 to 1.6 per 100,000/year
Prevalence: 0.8 to 8.9 per 100,000/year
Median age of onset: 4...

Clinical Burden of CIDP
IVIg = intravenous immunoglobulin.
Querol et al, 2021; Van Schaik et al, 2010.
Treatments can be c...

Clinical Burden of CIDP (cont.)
Patients experience physical and psychosocial burdens, including
impaired physical functio...

Economic Burden of CIDP
Querol et al, 2021.
Distribution of CIDP direct costs
by cost components in the UK and
USA. USA co...

Unmet Treatment Needs in CIDP
Querol et al, 2021.
Diagnostic delay
Clinical consequences
Increased disability
Worse long-t...

Patient Case: Erik

Patient Case: Erik
Erik is a 49-year-old man who is experiencing weakness and
tingling in his legs and hands, with difficu...

Patient Case: Erik (cont.)
He noticed symptoms of difficulty climbing stairs and imbalance
with walking
He also began to n...

Patient Case: Erik (cont.)
PMH = past medical history.
PMH: lumbar spinal stenosis, pre-diabetes
Medications: none
Impact ...

Mastering the Complexities of CIDP Diagnosis
Van den Bergh et al, 2021.
Typical CIDP presents with progressive numbness an...

NCS = nerve conduction studies; MADSAM = multifocal acquired demyelinating sensory and motor neuropathy.
Van den Bergh et ...

Patient Case: Erik (cont.)
So, is it clear at this point that Erik has CIDP?
a. Yes
b. No

Patient Case: Erik (cont.)
So, is it clear at this point that Erik has CIDP?
a. Yes
b. No
No, because the timeline of prog...

Patient Case: Erik (cont.)
SA = shoulder abductors; EE = elbow extensors; EF = elbow flexors; WE = wrist extensors; FE = f...

Patient Case: Erik (cont.)
Erik is diagnosed with GBS, gets treated with 2 g/kg IVIg
split over 5 days, and tolerates it w...

Patient Case: Erik (cont.)
Still within the 8-week window of time, it could be a treatment-
related fluctuation (TRF) of G...

Patient Case: Erik (cont.)
Now beyond 8 weeks of symptoms with frequent TRFs
Is this still GBS, or could it be acute-onset...

Patient Case: Erik (cont.)
Leonhard et al, 2010; Ruts et al, 2010.
Now beyond 8 weeks of symptoms with frequent TRFs
Is th...

Patient Case: Erik (cont.)
Diagnosis adjusted: CIDP
Clinical phenotype: typical
Next step in diagnosis: evaluating the ele...

Nerve Conduction Criteria
ULN = upper limit of normal; LLN = lower limit of normal; CMAP = compound action muscle potentia...

Nerve Conduction Criteria (cont.)
SNAP = sensory nerve action potential.
Van den Bergh et al, 2021.
(1) CIDP
• Sensory con...

NCS: Features Seen in CIDP
Conduction block
Temporal dispersion
Prolonged distal latency
Reduced conduction velocity (demy...

Patient Case: Erik (cont.)
R = right; dis = distal; prox = proximal; L = left; med = median nerve; rad = radial nerve; sup...

Supportive Criteria for the Diagnosis of CIDP
Van den Bergh et al, 2021
Response to treatment
Outcome measures are importa...

Supportive Criteria: Response to Treatment
MRC = Medical Research Council; INCAT = Inflammatory Neuropathy Cause and Treat...

Metrics to Assess Response to Treatment
SE = standard error; MCID = minimally clinically important difference.
Allen et al...

CSF Albuminocytological Dissociation
• Caution with mild or moderate CSF protein elevation, especially
in the presence of ...

Supportive Criteria: Imaging
MRI = magnetic resonance imaging.
Van den Bergh et al, 2021.
Ultrasound Criteria
*Cross-secti...

Supportive Criteria: Nerve Biopsy
Van den Bergh et al, 2021.
Rarely needed
Features:
Thinly myelinated axons and small oni...

Game: What Do These Symptoms Suggest?
i3 Health, 2025.
Suggest CIDP in differential diagnosis
if symptoms present for grea...

Results: What Do These Symptoms Suggest?
i3 Health, 2025.

Differential Diagnosis: Red Flags That Suggest Another Diagnosis
CMT = Charcot-Marie-Tooth disease; ATTRv = hereditary tra...

Consider Further Investigation of Variants
ESR = erythrocyte sedimentation rate; anti-GM1 = anti-ganglioside M1; NMJ = neu...

Strategies to Avoid Common Pitfalls in Diagnosis
Van den Bergh et al, 2021.
Strategy: clinical phenotype + electrodiagnost...

EAN/PNS Guidelines for Diagnosis of CIDP
European Academy of Neurology; PNS = Peripheral Nerve Society; PLEX = plasma exch...

Game: Diagnostic Detective
Card
1
Card
2
Card
3
Card
4
Card
5
Scan the QR code or head over to
i3health.com/cidp to partic...

Results: Diagnostic Detective
Card
1
Card
2
Card
3
Card
4
Card
5
i3 Health, 2025.

CIDP: Optimizing Induction
and Maintenance Therapy

EAN/PNS Guidelines 2021 on Treatment of CIDP
EFNS = European
Federation of
Neurological
Societies.
Doneddu et al,
2022; Va...

Objective
improvement
Com
plete
response
Partial
response
If appears
inactive
No objective improvement
X
Still CIDP?
CIDP ...

CIDP Treatment: Corticosteroids
Austin et al, 1958; Dyck et al, 1982.
In 1958, Austin reported that some recurrent polyneu...

CIDP Treatment: Corticosteroids (cont.)
QOD = once daily.
Dyck et al, 1982.
Objective: compare corticosteroids
to no treat...

CIDP Treatment: Corticosteroids (cont.)
PREDICT Study (2010)
Objective: pulsed oral dexamethasone
vs oral prednisolone
Des...

CDAS Status N (%) Follow-Up
Lost to follow up 1
Died 6
Alternative diagnosis 7 (18%)
Amyloid, hereditary,
lymphoma
CDAS cu...

CIDP Treatment: Corticosteroids (cont.)
Van den Bergh et al, 2021; Eftimov et al, 2012.
Summary of Data
Quality evidence s...

CIDP Treatment: IVIg
Summary of Data
Quality evidence supporting IVIg
for CIDP is high
In total, 5 randomized controlled
s...

Corticosteroids vs IVIg Trial 2012
IV = intravenous;
MP =
methylprednisolo
ne.
Nobile-Orazio et
al, 2012.
Objective
Methyl...

Corticosteroids vs IVIg Trial 2014: Follow-Up
Nobile-Orazio et al, 2015.
Efficacy
IVIg: 28 of 32 improved (87.5%)
MP: 13 o...

Conclusions: Corticosteroids vs IVIg
Nobile-Orazio et al, 2012; Nobile-Orazio et al, 2015; Eftimov et al, 2013.
IVIg and c...

IVIg and Steroid Treatment: Risks and Benefits
Stiehm, 2013; Koshi et al, 2022; Wasserman, 2008.
Potential side effects of...

CIDP Treatment: Plasma Exchange (PLEX)
Dyck et al, 1986; Hahn et al, 1996; Dyck et al, 1994.
PLEX vs Placebo
2 small trial...

Conclusions: PLEX for CIDP
Mehndiratta et al, 2015.
Moderate-to-high–quality evidence shows that PLEX provides
significant...

PLEX Treatment: Potential Complications
Sutton et al, 1989; Lu et al, 2019.
Allergic reactions or anaphylaxis
Pruritus and...

CIDP Treatment: PATH Trial SCIg
Van Schaik et al, 2018.
Objective: SCIg vs placebo
Design: randomized double-blind,
placeb...

CIDP Treatment: SCIg ADVANCE-CIDP Trial
Randomized, double-blind, placebo-controlled
phase 3 trial evaluating the efficacy...

Anti-FcRn Class
HCP = healthcare provider.
Allen et al, 2024; FDA, 2024.
FDA-approved for CIDP: efgartigimod
ADHERE study ...

CIDP-Targeted Therapy
FcRn = neonatal
fragment
crystallizable
receptor.
Gable & Guptil,
2020.
Anti-FcRn Treatments
FcRn-me...

Phase 2 multicenter, randomized, quadruple-blind, placebo-controlled trial in patients with CIDP
Patient selection
• CIDP ...

0
10
20
30
40
50
60
70
80
90
100
66.5% 70.4%
Percent
of
Participants
With
Confirmed
improvement
Participants
who reached
e...

Game: What Treatment Would You Recommend?
i3 Health, 2025.
Initial Therapy Maintenance Therapy
Not Recommended Therapy
Mat...

Results: What Treatment Would You Recommend?
i3 Health, 2025.

Emerging Agents: Ongoing FcRn Clinical Trial
CS = corticosteroids;
CISP = chronic immune sensory polyneuropathy; Q2W = eve...

Emerging Agents: FcRn Clinical Trial
QW = every week.
NCT05581199; Immunovant, 2022.
IMVT-1401-2401 Trial
Study
Timeline
W...

Emerging Agents: FcRn Class
NCT07032662.
A phase 2b, multicenter, randomized, double-blind, placebo-
controlled study of I...

Emerging CIDP Therapies
MBL = mannose-
binding lectin;
MAC = membrane
attack complex.
Gorson et al, 2010;
Bus et al, 2022;...

Emerging Agents: Complement Inhibition
C = complement.
Berentsen & Sundic, 2015; NCT06290141; NCT06290128; NCT06920004; NC...

Emerging Agents
CD = cluster of differentiation.
Forsthuber et al, 2017; Rituxan®
prescribing information, 2021.
Summary o...

Randomized controlled trial of rituximab vs placebo in CIDP
Location: Italian centers
Purpose: does rituximab prevent wors...

Switching From IVIg to SCIg: Outcomes
MMN = multifocal motor neuropathy.
Kapoor et al, 2021.
47/56 patients (CIDP & MMN) w...

FcRn Studies
Allen et al, 2024; Levine & Muley, 2025.
Efgartigimod
Effective at improving disability, improving strength, ...

Switching From IVIg to FcRn
NCT06637072.
Open-label study ongoing to evaluate this strategy
Would recommend following simi...

Strategies for Personalizing Treatment
Slide courtesy of Karissa Gable, MD.
Patient-centered care is key to therapeutic su...

Evolving Treatment Landscape
Personalizing treatment:
Depends on comorbidities of patient
Route of administration preferen...

Patient Case: Erik (cont.)
Erik has been regularly receiving 1g/kg IVIg every 3 weeks for
3 cycles now
He is seen in follo...

Outcome Measures
MGUSP = monoclonal gammopathy
of undetermined significance polyneuropathy;
RT-mISS = Rasch-Transformed
Mo...

Metrics
Alllen et al, 2021.
Frequently Used Definitions of MCID in CIDP
Scale MCID Remarks
(Adjusted) INCAT 1 point
• Not ...

Patient Case: Erik (cont.)
3 months after regular treatment with 1g/kg IVIg, Erik demonstrates the
following changes on ex...

Patient Case: Erik (cont.)
After waiting until the patient reaches a plateau of improvement
with stability for 6 months
St...

Patient Case: Erik (cont.)
The patient expresses some issues with side effects with IVIg
Pre-medications make him tired; h...

Key Takeaways
PT = physical therapy; OT = occupational therapy.
Set goals and expectations for treatment; consider comorbi...

References
Allen JA, Eftimov F & Querol L (2021). Outcome measures and biomarkers in chronic inflammatory demyelinating po...

References (cont.)
Clinicaltrials.gov (2025). A study to test the efficacy and safety of riliprubart against the usual tre...

References (cont.)
Hughes RAC, Donofrio P, Bril V, et al (2008). Intravenous immune globulin (10% caprylate-chromatography...

References (cont.)
Querol L, Lewis RA, Hartung HP, et al (2024). Phase 2 efficacy and safety of riliprubart, a C1s-complem...

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