Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)¶
VERSION: 1.0 CREATED: January 27, 2026 REVISED: January 27, 2026 STATUS: Draft - Pending Review
DIAGNOSIS: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
ICD-10: G61.81
SCOPE: Evaluation, diagnosis, treatment initiation, and long-term management of CIDP in adults. Covers typical CIDP, CIDP variants (MADSAM, DADS, sensory CIDP), first-line immunotherapy (IVIg, corticosteroids, plasmapheresis), second-line immunosuppressants, and subcutaneous immunoglobulin therapy. Primary focus is outpatient management with coverage for acute presentations and hospitalizations. Excludes Guillain-Barré syndrome (acute onset <8 weeks), hereditary neuropathies (CMT), and pediatric CIDP.
CLINICAL SYNONYMS: Chronic inflammatory demyelinating polyradiculoneuropathy, chronic relapsing polyneuropathy, chronic acquired demyelinating polyneuropathy, CIDP
ADDITIONAL ICD-10 CODES: - G61.89 - Other inflammatory polyneuropathies - G62.9 - Polyneuropathy, unspecified (pre-diagnosis)
KEY CLINICAL FEATURES: - Progressive or relapsing symmetric proximal AND distal weakness - Sensory symptoms (numbness, paresthesias, sensory ataxia) - Hyporeflexia or areflexia - Duration >8 weeks (distinguishes from GBS) - Elevated CSF protein with normal cell count (albuminocytologic dissociation) - Electrodiagnostic evidence of demyelination
CIDP VARIANTS: | Variant | Features | Notes | |---------|----------|-------| | Typical CIDP | Symmetric proximal and distal weakness, sensory loss | Most common (~50%) | | MADSAM (Lewis-Sumner) | Multifocal, asymmetric; sensorimotor | May mimic MMN | | DADS | Distal acquired demyelinating symmetric; sensory predominant | Often anti-MAG positive | | Sensory CIDP | Pure sensory; ataxia, large fiber loss | NCS shows demyelination | | Motor CIDP | Pure motor; no sensory involvement | Rare; consider MMN | | Focal CIDP | Involvement of one or two limbs | Rare |
EFNS/PNS DIAGNOSTIC CRITERIA (2021 Update):
Clinical Criteria: - Typical CIDP: Progressive or relapsing symmetric proximal and distal weakness AND sensory dysfunction of all extremities, developing over ≥8 weeks - Cranial nerves may be affected - Tendon reflexes reduced or absent in all limbs
Supportive Criteria: - CSF protein elevated with WBC <10/mm³ - MRI showing nerve root or plexus enhancement or hypertrophy - Objective clinical improvement following immunomodulatory treatment
PRIORITY KEY: STAT = Immediate | URGENT = Within hours | ROUTINE = Standard | EXT = Extended/atypical cases | - = Not applicable to this setting
📋 CHRONIC DISEASE NOTE
CIDP is a chronic, treatable condition. Most patients respond to first-line immunotherapy. The goal is symptom control with minimal treatment burden. Long-term monitoring and periodic treatment adjustments are essential.
═══════════════════════════════════════════════════════════════ SECTION A: ACTION ITEMS ═══════════════════════════════════════════════════════════════
1. LABORATORY WORKUP¶
1A. Essential/Core Labs¶
| Test | Rationale | Target Finding | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|
| CBC with differential | Baseline, infection screen, pre-treatment | Normal | STAT | STAT | ROUTINE | STAT |
| CMP (BMP + LFTs) | Electrolytes, renal/hepatic function for IVIg | Normal | STAT | STAT | ROUTINE | STAT |
| ESR | Inflammatory marker; elevated in some CIDP | Document baseline | URGENT | ROUTINE | ROUTINE | URGENT |
| CRP | Inflammatory marker | Document baseline | URGENT | ROUTINE | ROUTINE | URGENT |
| HbA1c | Diabetic neuropathy in differential; diabetes worsens CIDP | <5.7% (normal); >6.5% diagnostic for diabetes | URGENT | ROUTINE | ROUTINE | URGENT |
| Fasting glucose | Diabetes screening | <100 mg/dL | URGENT | ROUTINE | ROUTINE | URGENT |
| TSH | Thyroid disease causing neuropathy | Normal (0.4-4.0 mIU/L) | URGENT | ROUTINE | ROUTINE | URGENT |
| Vitamin B12 | B12 deficiency neuropathy | >300 pg/mL | URGENT | ROUTINE | ROUTINE | URGENT |
| Methylmalonic acid | If B12 borderline (200-400 pg/mL) | Normal if B12 sufficient | — | ROUTINE | ROUTINE | — |
| Serum protein electrophoresis (SPEP) | Paraproteinemic neuropathy screen | No monoclonal spike | — | ROUTINE | ROUTINE | — |
| Serum immunofixation | Confirms monoclonal protein if SPEP abnormal | Characterize M-protein | — | ROUTINE | ROUTINE | — |
| HIV antibody | HIV-associated neuropathy/CIDP | Negative | — | ROUTINE | ROUTINE | — |
| Hepatitis B surface antigen | Pre-IVIg screening; HBV reactivation risk | Negative | — | URGENT | ROUTINE | URGENT |
| Hepatitis B core antibody | Prior HBV exposure; reactivation risk with immunosuppression | Negative | — | URGENT | ROUTINE | URGENT |
| Hepatitis C antibody | HCV-associated cryoglobulinemic neuropathy | Negative | — | ROUTINE | ROUTINE | — |
1B. Extended Workup (Second-line)¶
| Test | Rationale | Target Finding | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|
| Urine protein electrophoresis (UPEP) | Light chain deposition in neuropathy | No monoclonal protein | — | ROUTINE | ROUTINE | — |
| Serum free light chains | AL amyloidosis, POEMS screening | Normal kappa:lambda ratio | — | ROUTINE | ROUTINE | — |
| VEGF level | Elevated in POEMS syndrome | Normal (<200 pg/mL) | — | EXT | ROUTINE | — |
| Anti-MAG antibody | DADS variant; IgM paraproteinemic neuropathy | Negative (if positive, suggests DADS-CIDP or anti-MAG neuropathy) | — | ROUTINE | ROUTINE | — |
| Anti-ganglioside antibodies (GM1, GD1a, GD1b) | MMN differentiation; axonal GBS variants | Document if positive | — | ROUTINE | ROUTINE | — |
| Anti-GQ1b antibody | Miller Fisher variant if ophthalmoplegia | Document if positive | — | ROUTINE | ROUTINE | — |
| Quantitative immunoglobulins (IgG, IgA, IgM) | IgA deficiency (IVIg contraindication); IgM elevation | IgA >7 mg/dL; document IgM | — | URGENT | ROUTINE | URGENT |
| ANA | Connective tissue disease screen | Negative or low titer | — | ROUTINE | ROUTINE | — |
| Rheumatoid factor | Vasculitic neuropathy screen | Negative | — | ROUTINE | ROUTINE | — |
| Anti-SSA/SSB | Sjogren syndrome neuropathy | Negative | — | ROUTINE | ROUTINE | — |
| ACE level | Sarcoid neuropathy | Normal | — | EXT | ROUTINE | — |
| PT/INR, PTT | Pre-LP, pre-PLEX | Normal | URGENT | ROUTINE | — | STAT |
| Type and screen | Pre-PLEX | Available | — | URGENT | — | STAT |
1C. Rare/Specialized (Refractory or Atypical)¶
| Test | Rationale | Target Finding | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|
| Anti-contactin-1 (CNTN1) antibody | Aggressive CIDP variant; poor IVIg response | Document if positive | — | EXT | EXT | — |
| Anti-neurofascin-155 (NF155) antibody | Young onset, tremor, poor IVIg response | Document if positive | — | EXT | EXT | — |
| Anti-caspr-1 antibody | Nodopathy; aggressive course | Document if positive | — | EXT | EXT | — |
| Anti-NF186/NF140 antibodies | Nodal/paranodal antibodies | Document if positive | — | EXT | EXT | — |
| Genetic testing for CMT | If family history or atypical features | Negative (excludes CMT) | — | EXT | EXT | — |
| Lyme serology | Endemic area, outdoor exposure | Negative | — | EXT | ROUTINE | — |
| Heavy metals (lead, arsenic) | Toxic neuropathy differential | Normal | — | EXT | EXT | — |
| Urine porphyrins | Motor neuropathy with systemic symptoms | Normal | — | EXT | EXT | — |
| Bone marrow biopsy | If POEMS or amyloidosis suspected | Normal or diagnostic | — | EXT | EXT | — |
| Fat pad biopsy for amyloid | If amyloidosis suspected | Negative | — | EXT | EXT | — |
| Cryoglobulins | If HCV positive or vasculitic features | Negative | — | EXT | ROUTINE | — |
2. DIAGNOSTIC IMAGING & STUDIES¶
2A. Essential/First-line¶
| Study | Timing | Target Finding | Contraindications | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|
| Nerve conduction studies (NCS) | At diagnosis; before treatment | Demyelinating features: prolonged distal latencies (>125% ULN), slow conduction velocities (<80% LLN), conduction block, temporal dispersion, prolonged F-waves | None | — | URGENT | ROUTINE | — |
| Electromyography (EMG) | At diagnosis; with NCS | Secondary axonal loss; fibrillations in severe cases | Severe coagulopathy | — | URGENT | ROUTINE | — |
| Chest X-ray | Pre-treatment baseline | Normal (rule out mass if paraneoplastic concern) | None | STAT | ROUTINE | ROUTINE | STAT |
2B. Extended¶
| Study | Timing | Target Finding | Contraindications | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|
| MRI spine with contrast (cervical/lumbar) | Diagnostic workup | Nerve root enhancement, cauda equina hypertrophy | Pacemaker, severe claustrophobia | — | ROUTINE | ROUTINE | — |
| MRI brachial plexus with contrast | If asymmetric or plexus involvement | Nerve hypertrophy, enhancement | Pacemaker | — | ROUTINE | ROUTINE | — |
| MRI lumbosacral plexus with contrast | If lower extremity predominant | Plexus enhancement, thickening | Pacemaker | — | ROUTINE | ROUTINE | — |
| Nerve ultrasound | Complementary to MRI; assess nerve enlargement | Increased cross-sectional area of peripheral nerves | None | — | ROUTINE | ROUTINE | — |
| CT chest/abdomen/pelvis | If POEMS or malignancy suspected | Sclerotic bone lesions (POEMS), organomegaly | Contrast allergy, renal impairment | — | EXT | ROUTINE | — |
2C. Rare/Specialized¶
| Study | Timing | Target Finding | Contraindications | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|
| PET-CT | Occult malignancy, lymphoma, Castleman disease | Tumor identification | Hemodynamic instability | — | EXT | EXT | — |
| Nerve biopsy (sural) | Diagnostic uncertainty, vasculitis suspected | Demyelination, inflammation, onion bulbs | Active infection at site | — | EXT | EXT | — |
| Skeletal survey | POEMS evaluation | Sclerotic or lytic lesions | None | — | EXT | ROUTINE | — |
LUMBAR PUNCTURE¶
Indication: Confirm diagnosis (albuminocytologic dissociation); exclude infection or malignancy; required for EFNS/PNS supportive criteria
Timing: ROUTINE for diagnosis; not urgent unless acute deterioration or infection concern
Volume Required: 10-15 mL standard diagnostic
| Study | Rationale | Target Finding | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|
| Opening pressure | Baseline; usually normal in CIDP | 10-20 cmH2O | URGENT | ROUTINE | ROUTINE | — |
| Cell count (tubes 1 and 4) | Albuminocytologic dissociation | WBC <10/mm³ (usually <5) | URGENT | ROUTINE | ROUTINE | — |
| Protein | Elevated in CIDP | Elevated (>45 mg/dL; often 50-200 mg/dL) | URGENT | ROUTINE | ROUTINE | — |
| Glucose | Exclude infection | Normal (>60% serum glucose) | URGENT | ROUTINE | ROUTINE | — |
| Gram stain and culture | Exclude infection | Negative | URGENT | ROUTINE | — | — |
| Cytology | Exclude carcinomatous meningitis | Negative | — | ROUTINE | ROUTINE | — |
| VDRL | Neurosyphilis in differential | Negative | — | ROUTINE | ROUTINE | — |
| Oligoclonal bands | MS or CNS inflammation in differential | Usually negative; may be mildly positive | — | ROUTINE | ROUTINE | — |
Special Handling: Cell count within 1 hour. Cytology refrigerated.
Contraindications: Coagulopathy (INR >1.5, platelets <50K), anticoagulation, skin infection at LP site.
NOTE: CSF protein is elevated in >80% of CIDP patients. Normal CSF protein does not exclude CIDP if electrodiagnostic criteria are met.
3. TREATMENT¶
CRITICAL: CIDP is treatable. First-line therapies (IVIg, corticosteroids, PLEX) are effective in ~80% of patients. Treatment choice depends on patient characteristics, access, and preference.
Treatment Selection Guidance¶
| Factor | IVIg Preferred | Corticosteroids Preferred | PLEX Preferred |
|---|---|---|---|
| Pure motor CIDP | Yes | Avoid (may worsen motor) | Yes |
| Diabetes mellitus | Yes | Avoid (worsens glycemic control) | Yes |
| Cost/access concerns | If covered | Yes (low cost) | Limited access |
| Rapid response needed | Yes | No (slow onset) | Yes |
| Long-term therapy | Maintenance possible | Taper to lowest dose; add steroid-sparing | Not practical |
| Pregnancy | Preferred | Avoid if possible | Safe |
| Nodal/paranodal antibodies | May be less effective | May be effective | May be effective; rituximab preferred |
3A. First-line Immunotherapy - Induction¶
| Treatment | Route | Indication | Dosing | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|
| IVIg (immune globulin IV) - Loading | IV | First-line induction | 2 g/kg over 2-5 days :: IV :: divided over 2-5 days :: 2 g/kg total divided over 2-5 days (e.g., 0.4 g/kg/day x 5 days or 1 g/kg/day x 2 days); infuse slowly day 1 (start 0.5-1 mL/kg/hr, max 4 mL/kg/hr) | IgA deficiency with anti-IgA antibodies; uncontrolled heart failure; recent thrombosis; severe renal impairment | Renal function, headache, infusion reactions; pre-medicate with acetaminophen and diphenhydramine | — | URGENT | ROUTINE | URGENT |
| Prednisone - Daily | PO | First-line induction (if IVIg unavailable or contraindicated) | 60 mg daily; 1 mg/kg daily :: PO :: daily :: Start 60 mg (or 1 mg/kg) daily for 4-8 weeks until clinical improvement, then begin slow taper | Uncontrolled diabetes, active infection, psychosis, osteoporosis | Glucose, BP, bone density, mood, infection signs | — | ROUTINE | ROUTINE | — |
| Prednisone - Pulse | PO | Alternative oral pulse therapy | 40 mg daily x 5 days monthly :: PO :: daily x 5 days q4wk :: 40-60 mg daily for 5 consecutive days each month; may have fewer side effects than daily | Uncontrolled diabetes, active infection | Glucose, BP during pulse | — | ROUTINE | ROUTINE | — |
| Methylprednisolone - IV Pulse | IV | Severe CIDP, rapid induction needed | 1000 mg daily x 3-5 days :: IV :: daily x 3-5 days :: 1000 mg IV daily for 3-5 days; can repeat monthly as needed; transition to oral | Active untreated infection, uncontrolled HTN/diabetes | Glucose, BP, K+, psychiatric effects | — | URGENT | ROUTINE | URGENT |
| Plasma exchange (PLEX) | — | First-line if IVIg/steroids contraindicated or failed | 5-10 exchanges over 2-4 weeks :: — :: 2-3x/week :: 5-10 exchanges (1-1.5 plasma volumes each) over 2-4 weeks; typically 2-3 sessions per week | Hemodynamic instability, severe sepsis, poor vascular access | BP, electrolytes, coagulation, fibrinogen | — | URGENT | — | URGENT |
3B. First-line Immunotherapy - Maintenance¶
| Treatment | Route | Indication | Dosing | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|
| IVIg - Maintenance | IV | Maintenance after loading response | 0.4 g/kg q3wk; 0.5 g/kg q3wk; 1 g/kg q3-4wk :: IV :: q3-4wk :: 0.4-1 g/kg every 3-4 weeks; titrate to lowest effective dose and longest interval; some patients need 1 g/kg q2-3wk | IgA deficiency, renal impairment, recent thrombosis | Renal function q3-6mo; trough IgG levels optional | — | ROUTINE | ROUTINE | — |
| SCIg (Hizentra) - Maintenance | SC | Home maintenance after IVIg response | Weekly dose = IVIg monthly dose ÷ 4 :: SC :: weekly or divided :: Convert from IVIg: weekly SC dose equals monthly IV dose divided by 4; typical range 0.2-0.4 g/kg/week; divide into 1-7 infusions/week | Severe thrombocytopenia, skin conditions at injection site | Local site reactions; IgG trough levels q6mo | — | — | ROUTINE | — |
| SCIg (Gamunex-C 10%) - Maintenance | SC | Home maintenance alternative | Weekly dose = IVIg monthly dose ÷ 4 :: SC :: weekly :: Same as Hizentra; 10% concentration; can give larger volume per site | Severe thrombocytopenia | Local reactions, IgG levels | — | — | ROUTINE | — |
| Prednisone - Maintenance taper | PO | Continuation after steroid induction | Taper by 5-10 mg q2-4wk :: PO :: daily :: After improvement, taper by 5-10 mg every 2-4 weeks; target lowest effective dose (often 5-20 mg daily or QOD); add steroid-sparing agent if unable to taper below 15-20 mg | Ongoing infection | Glucose, BP, bone density, cataracts | — | ROUTINE | ROUTINE | — |
3C. Second-line/Steroid-Sparing Agents¶
| Treatment | Route | Indication | Dosing | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|
| Azathioprine | PO | Steroid-sparing; maintenance | 50 mg daily; 100 mg daily; 150 mg daily; 2-3 mg/kg daily :: PO :: daily :: Start 50 mg daily; increase by 50 mg every 2-4 weeks to target 2-3 mg/kg/day; check TPMT before starting; onset 3-6 months | TPMT deficiency, pregnancy, myelosuppression | CBC weekly x 4 weeks, then monthly x 3 months, then q3mo; LFTs monthly | — | — | ROUTINE | — |
| Mycophenolate mofetil | PO | Steroid-sparing; maintenance | 500 mg BID; 1000 mg BID; 1500 mg BID :: PO :: BID :: Start 500 mg BID; increase to 1000-1500 mg BID over 2-4 weeks; onset 2-4 months | Pregnancy (teratogenic), severe renal impairment | CBC q2wk x 2 months, then monthly; LFTs | — | — | ROUTINE | — |
| Rituximab | IV | Refractory CIDP, nodal antibody-positive | 375 mg/m² weekly x 4 weeks; 1000 mg q2wk x 2 :: IV :: weekly x 4 or q2wk x 2 :: 375 mg/m² weekly x 4 doses OR 1000 mg x 2 doses (2 weeks apart); repeat q6-12mo PRN; pre-medicate | Active infection, HBV (screen first; may reactivate) | Infusion reactions, CD19/CD20 counts, immunoglobulins; HBV DNA if prior exposure | — | EXT | EXT | — |
| Cyclophosphamide | IV | Severe refractory CIDP | 1 g/m² monthly x 6 months :: IV :: monthly :: 1 g/m² IV monthly for 6 months; reserve for severe refractory cases | Pregnancy, active infection, hemorrhagic cystitis | CBC, urinalysis, fertility counseling; MESNA for bladder protection | — | EXT | EXT | — |
| Cyclosporine | PO | Steroid-sparing alternative | 2.5-5 mg/kg/day divided BID :: PO :: BID :: Start 2.5 mg/kg/day divided BID; increase to 5 mg/kg/day based on levels and response; target trough 100-200 ng/mL | Uncontrolled HTN, renal impairment | Cr, BP, drug levels, K+, Mg | — | — | EXT | — |
3D. Disease-Modifying Therapy - Special Populations¶
| Treatment | Route | Indication | Dosing | Pre-Treatment Requirements | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|---|
| IVIg - Anti-MAG positive | IV | Anti-MAG neuropathy (may have partial response) | 2 g/kg loading then 0.4-1 g/kg q3-4wk :: IV :: per protocol :: Anti-MAG neuropathy often less responsive to IVIg; may require rituximab if poor response | SPEP/UPEP to assess for underlying plasma cell dyscrasia | IgA deficiency | Response may be slower; consider rituximab if no improvement | — | ROUTINE | ROUTINE | — |
| Rituximab - Anti-MAG positive | IV | Anti-MAG neuropathy preferred therapy | 375 mg/m² weekly x 4 weeks; 1000 mg q2wk x 2 :: IV :: weekly x 4 or q2wk x 2 :: First-line for anti-MAG neuropathy given superior efficacy; repeat q6-12mo | HBV/HCV screening, quantitative immunoglobulins, CBC | Active HBV, active infection | Anti-MAG titers, CD19/20, immunoglobulins | — | EXT | ROUTINE | — |
| Rituximab - Nodal antibody positive (NF155, CNTN1) | IV | CIDP with nodal/paranodal antibodies | 375 mg/m² weekly x 4 weeks; 1000 mg q2wk x 2 :: IV :: weekly x 4 or q2wk x 2 :: Preferred for NF155/CNTN1/CASPR1 positive CIDP (poor IVIg response); repeat q6mo | HBV/HCV screening, immunoglobulins | Active HBV | Antibody titers, B-cell counts | — | EXT | ROUTINE | — |
3E. Symptomatic Treatments¶
| Treatment | Route | Indication | Dosing | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|
| Gabapentin | PO | Neuropathic pain | 300 mg qHS; 300 mg TID; 600 mg TID; 900 mg TID :: PO :: TID :: Start 300 mg qHS; titrate by 300 mg/day q3d; target 900-1800 mg TID; max 3600 mg/day | Severe renal impairment (adjust dose) | Sedation, dizziness, edema; reduce dose if CrCl <60 | — | ROUTINE | ROUTINE | — |
| Pregabalin | PO | Neuropathic pain | 75 mg BID; 150 mg BID; 300 mg BID :: PO :: BID :: Start 75 mg BID; increase to 150 mg BID after 1 week; max 600 mg/day | Severe renal impairment (adjust dose) | Sedation, edema, weight gain | — | ROUTINE | ROUTINE | — |
| Duloxetine | PO | Neuropathic pain, depression | 30 mg daily; 60 mg daily :: PO :: daily :: Start 30 mg daily; increase to 60 mg daily after 1-2 weeks; max 120 mg/day | Uncontrolled glaucoma, severe hepatic impairment, MAOIs | BP, hepatic function, serotonin syndrome | — | ROUTINE | ROUTINE | — |
| Amitriptyline | PO | Neuropathic pain, sleep | 10 mg qHS; 25 mg qHS; 50 mg qHS; 75 mg qHS :: PO :: qHS :: Start 10 mg qHS; titrate by 10-25 mg q1-2wk; max 150 mg qHS | Cardiac arrhythmias, recent MI, glaucoma, urinary retention | ECG if >100 mg/day; anticholinergic effects | — | ROUTINE | ROUTINE | — |
| Acetaminophen | PO | Mild pain | 650 mg q6h PRN; 1000 mg q6h PRN :: PO :: q6h PRN :: 650-1000 mg q6h PRN; max 4 g/day (3 g if hepatic impairment) | Severe hepatic impairment | LFTs if prolonged use | STAT | ROUTINE | ROUTINE | STAT |
| Mexiletine | PO | Muscle cramps, neuropathic pain (refractory) | 150 mg TID; 200 mg TID :: PO :: TID :: Start 150 mg TID; may increase to 200-300 mg TID; max 1200 mg/day | Cardiac arrhythmia, heart block | ECG, hepatic function | — | EXT | EXT | — |
| Baclofen | PO | Muscle cramps, spasms | 5 mg TID; 10 mg TID; 20 mg TID :: PO :: TID :: Start 5 mg TID; increase by 5 mg/dose every 3 days; max 80 mg/day | Severe renal impairment | Sedation; do not stop abruptly | — | ROUTINE | ROUTINE | — |
3F. Prophylaxis and Supportive Care¶
| Treatment | Route | Indication | Dosing | Contraindications | Monitoring | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|---|---|
| Calcium + Vitamin D | PO | Bone protection on steroids | Calcium 1000-1200 mg daily; Vitamin D 800-2000 IU daily :: PO :: daily :: Calcium 500-600 mg BID (with food) + Vitamin D 800-2000 IU daily for all patients on chronic steroids | Hypercalcemia, kidney stones | Calcium levels, 25-OH vitamin D | — | ROUTINE | ROUTINE | — |
| Bisphosphonate (alendronate) | PO | Osteoporosis prevention on steroids | 70 mg weekly :: PO :: weekly :: 70 mg PO weekly if prednisone >7.5 mg for >3 months; take with water, remain upright 30 min | Esophageal disorders, CrCl <35, hypocalcemia | DEXA scan at baseline and q1-2yr; dental exam | — | — | ROUTINE | — |
| Omeprazole | PO | GI protection on steroids | 20 mg daily :: PO :: daily :: 20 mg daily for patients on high-dose steroids or with GI symptoms | Long-term use concerns (Mg, B12, fractures) | Mg levels if prolonged | — | ROUTINE | ROUTINE | — |
| Aspirin (low-dose) | PO | Thrombosis prevention with IVIg | 81 mg daily :: PO :: daily :: 81 mg daily during IVIg infusions for patients with thrombosis risk factors (age >60, obesity, immobility, prior VTE, hyperviscosity) | Active bleeding, allergy | None routine | — | ROUTINE | ROUTINE | — |
| Enoxaparin | SC | DVT prophylaxis (immobile patients) | 40 mg daily :: SC :: daily :: 40 mg SC daily for immobile hospitalized patients; continue until ambulating | Active bleeding, HIT, CrCl <30 | Platelet count | — | STAT | — | STAT |
| PCP prophylaxis (TMP-SMX) | PO | Pneumocystis prevention on high-dose steroids | 1 DS tablet 3x/week :: PO :: 3x/week :: TMP-SMX DS (160/800 mg) 3x/week or daily if prednisone >20 mg for >4 weeks + another immunosuppressant | Sulfa allergy (use dapsone or atovaquone) | CBC, renal function | — | ROUTINE | ROUTINE | — |
4. OTHER RECOMMENDATIONS¶
4A. Referrals & Consults¶
| Recommendation | ED | HOSP | OPD | ICU |
|---|---|---|---|---|
| Neurology consult for diagnosis confirmation, treatment initiation, and long-term management planning | URGENT | STAT | — | STAT |
| Neuromuscular specialist referral for diagnostic uncertainty, treatment-refractory cases, or consideration of advanced therapies | — | ROUTINE | ROUTINE | — |
| Infusion center referral for ongoing IVIg administration and monitoring | — | ROUTINE | ROUTINE | — |
| Home infusion service referral for SCIg training and home therapy setup | — | ROUTINE | ROUTINE | — |
| Physical therapy for gait training, fall prevention, strengthening exercises, and mobility optimization | — | URGENT | ROUTINE | — |
| Occupational therapy for ADL assessment, adaptive equipment, and energy conservation strategies | — | URGENT | ROUTINE | — |
| Pain management consult for refractory neuropathic pain not responding to gabapentin/pregabalin/duloxetine | — | ROUTINE | ROUTINE | — |
| Endocrinology consult for steroid-induced diabetes management or osteoporosis prevention | — | ROUTINE | ROUTINE | — |
| Hematology consult if monoclonal protein detected for POEMS or myeloma workup | — | ROUTINE | ROUTINE | — |
| Pulmonology consult if respiratory symptoms develop (rare in CIDP; consider GBS overlap) | URGENT | URGENT | — | STAT |
| Rheumatology consult if connective tissue disease suspected based on labs or clinical features | — | ROUTINE | ROUTINE | — |
| Social work for disability resources, transportation assistance, and community support services | — | ROUTINE | ROUTINE | — |
| PCP follow-up for chronic disease management, steroid side effect monitoring, and preventive care | — | ROUTINE | ROUTINE | — |
4B. Patient Instructions¶
| Recommendation | ED | HOSP | OPD |
|---|---|---|---|
| Return immediately if progressive weakness, difficulty walking, or breathing problems develop (may indicate disease progression or acute worsening) | STAT | — | STAT |
| Keep all IVIg/infusion appointments as scheduled to maintain disease control and prevent relapses | — | ROUTINE | ROUTINE |
| Do not stop immunosuppressive medications suddenly as this may cause disease flare; taper only under physician guidance | STAT | STAT | STAT |
| Report signs of infection (fever, cough, dysuria) promptly as immunosuppression increases infection risk | STAT | STAT | STAT |
| Use fall precautions including handrails, non-slip surfaces, and assistive devices due to balance and weakness | STAT | STAT | STAT |
| Perform home exercises as prescribed by physical therapy to maintain strength and flexibility | — | ROUTINE | ROUTINE |
| Monitor for IVIg side effects including headache, fever, chills, or rash during infusions; report severe symptoms | — | ROUTINE | ROUTINE |
| Keep a symptom diary tracking strength, numbness, and functional abilities to share at follow-up visits | — | ROUTINE | ROUTINE |
| Carry a medical ID card indicating CIDP diagnosis and current treatments in case of emergency | — | ROUTINE | ROUTINE |
| Contact GBS-CIDP Foundation (www.gbs-cidp.org) for patient education, support groups, and resources | — | ROUTINE | ROUTINE |
4C. Lifestyle & Prevention¶
| Recommendation | ED | HOSP | OPD |
|---|---|---|---|
| Maintain glycemic control (HbA1c <7%) to prevent worsening of neuropathy if diabetic | — | ROUTINE | ROUTINE |
| Limit alcohol consumption as alcohol worsens peripheral neuropathy and interacts with medications | — | ROUTINE | ROUTINE |
| Ensure adequate vitamin B12 and folate intake through diet or supplementation to support nerve health | — | ROUTINE | ROUTINE |
| Avoid smoking as it impairs circulation and may worsen neuropathy | — | ROUTINE | ROUTINE |
| Stay up to date on vaccinations (flu, pneumonia, COVID-19, shingles) but avoid live vaccines while on immunosuppression | — | ROUTINE | ROUTINE |
| Wear protective footwear to prevent injury to numb feet and avoid burns or trauma | — | ROUTINE | ROUTINE |
| Perform daily foot inspections for wounds or ulcers given sensory loss | — | ROUTINE | ROUTINE |
| Engage in low-impact exercise (swimming, stationary cycling, walking) to maintain cardiovascular health and strength | — | ROUTINE | ROUTINE |
| Maintain bone health with calcium, vitamin D, and weight-bearing exercise, especially if on steroids | — | ROUTINE | ROUTINE |
| Manage stress and get adequate sleep as fatigue worsens CIDP symptoms | — | ROUTINE | ROUTINE |
═══════════════════════════════════════════════════════════════ SECTION B: REFERENCE (Expand as Needed) ═══════════════════════════════════════════════════════════════
5. DIFFERENTIAL DIAGNOSIS¶
| Alternative Diagnosis | Key Distinguishing Features | Tests to Differentiate |
|---|---|---|
| Guillain-Barré syndrome (GBS) | Acute onset (<4 weeks to nadir); monophasic; often post-infectious | Time course (<8 weeks); NCS; CSF |
| Multifocal motor neuropathy (MMN) | Pure motor; asymmetric; conduction block; no sensory involvement | Anti-GM1 antibodies; NCS (conduction block without sensory abnormality) |
| POEMS syndrome | Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes | VEGF, bone survey, SPEP/UPEP (lambda light chain) |
| Anti-MAG neuropathy | Distal, sensory-predominant; IgM paraprotein; tremor | Anti-MAG antibody; SPEP/UPEP |
| Hereditary neuropathy (CMT) | Family history; pes cavus, hammer toes; slowly progressive since childhood | Genetic testing; NCS pattern |
| Diabetic polyneuropathy | Length-dependent sensory > motor; diabetes history | HbA1c; NCS (axonal predominantly) |
| Vasculitic neuropathy | Painful; asymmetric; multifocal (mononeuritis multiplex); systemic symptoms | ESR/CRP, ANA, ANCA, nerve biopsy |
| Amyloid neuropathy | Autonomic involvement; carpal tunnel; cardiac involvement | Fat pad/nerve biopsy; genetic testing (TTR); serum free light chains |
| Lymphoma/paraneoplastic | Weight loss, lymphadenopathy; subacute course | CT/PET; paraneoplastic panel |
| Sarcoid neuropathy | Multisystem involvement; skin, lungs, lymph nodes | ACE level; chest imaging; biopsy |
| Paraproteinemic neuropathy (other) | IgG or IgA monoclonal; may mimic CIDP | SPEP/UPEP; bone marrow biopsy |
| Drug-induced neuropathy | Temporal relationship to drug; axonal pattern | Medication review; NCS |
| HIV-associated neuropathy | HIV risk factors; may have CSF pleocytosis | HIV testing; CD4 count |
| Lyme neuroborreliosis | Endemic area; facial palsy; radicular pain; CSF pleocytosis | Lyme serology; CSF Lyme antibodies |
6. MONITORING PARAMETERS¶
| Parameter | Frequency | Target/Threshold | Action if Abnormal | ED | HOSP | OPD | ICU |
|---|---|---|---|---|---|---|---|
| Clinical strength exam (MRC grading) | Each visit; q3-6mo stable | Stable or improved | Adjust therapy if declining | STAT | STAT | ROUTINE | STAT |
| INCAT disability score | Baseline and q6-12mo | Improvement or stability (0-10 scale) | Consider therapy change if worsening | — | ROUTINE | ROUTINE | — |
| ONLS (Overall Neuropathy Limitations Scale) | Baseline and q6-12mo | Improvement or stability (0-12 scale) | Consider therapy change if worsening | — | ROUTINE | ROUTINE | — |
| Grip strength (dynamometer) | Each visit | Stable or improved | Document trend; correlate with symptoms | — | ROUTINE | ROUTINE | — |
| Timed 10-meter walk | Baseline and q6-12mo | Stable or improved | Consider PT intensification if slowing | — | ROUTINE | ROUTINE | — |
| Nerve conduction studies | Baseline, 6mo after treatment, then annually if stable | Improvement or stability in demyelinating parameters | Guides treatment effectiveness | — | ROUTINE | ROUTINE | — |
| Renal function (BUN/Cr) | Before each IVIg; q3-6mo on IVIg | Cr stable (<1.5 or no rise) | Hold IVIg if rising; hydrate; may need dose adjustment | — | STAT | ROUTINE | STAT |
| CBC | q3mo on immunosuppressants | WBC >3K, ANC >1.5K, Hgb >10, Plt >100K | Hold azathioprine/MMF if cytopenic | — | ROUTINE | ROUTINE | — |
| LFTs | q3mo on immunosuppressants | ALT/AST <3x ULN | Hold azathioprine/MMF if significantly elevated | — | ROUTINE | ROUTINE | — |
| Blood glucose | Each visit on steroids; HbA1c q3mo | Fasting <126; HbA1c <7% | Intensify diabetes management | — | ROUTINE | ROUTINE | — |
| Blood pressure | Each visit on steroids | <140/90 (or <130/80 if diabetic) | Initiate antihypertensive if elevated | — | ROUTINE | ROUTINE | — |
| DEXA bone density | Baseline if steroids >3mo; repeat q1-2yr | T-score >-2.5 | Bisphosphonate if osteopenia/osteoporosis | — | — | ROUTINE | — |
| IgG trough level | q6mo on IVIg/SCIg (optional) | Trough >800-1000 mg/dL | Adjust dose if low and clinically worsening | — | — | ROUTINE | — |
| Anti-MAG titer | q6-12mo if positive | Declining with effective therapy | Guides treatment response for anti-MAG | — | — | EXT | — |
| CD19/CD20 B-cell count | After rituximab q3-6mo | Document depletion and reconstitution | Guides retreatment timing | — | — | EXT | — |
7. DISPOSITION CRITERIA¶
| Disposition | Criteria |
|---|---|
| Discharge home | Stable neurological exam; able to ambulate (with assistance if needed); oral medications tolerated; outpatient infusion arranged if needed; follow-up scheduled; adequate social support |
| Admit to hospital | Significant new weakness affecting ambulation; need for IV therapy initiation and monitoring; respiratory symptoms (rare but assess); unable to care for self at home |
| ICU admission | Respiratory compromise (FVC <30 mL/kg); rapidly progressive weakness raising concern for GBS overlap; severe autonomic dysfunction; hemodynamic instability |
| Outpatient infusion center | Stable CIDP on maintenance IVIg; appropriate for regular outpatient infusions every 3-4 weeks |
| Home infusion (SCIg) | Stable on IVIg who prefers home therapy; trained in self-injection; appropriate venous access not required |
| Rehabilitation facility | Significant residual weakness requiring intensive PT/OT; medically stable; goals for functional improvement |
8. EVIDENCE & REFERENCES¶
| Recommendation | Evidence Level | Source |
|---|---|---|
| IVIg effective for CIDP | Class I, Level A | Hughes et al. Lancet Neurol 2008 (ICE Trial) |
| IVIg dosing 2 g/kg loading, 1 g/kg maintenance | Class I, Level A | Hughes et al. Lancet Neurol 2008 (ICE Trial) |
| Corticosteroids effective for CIDP | Class I, Level A | Hughes et al. Cochrane 2017 |
| Plasma exchange effective for CIDP | Class I, Level A | Mehndiratta et al. Cochrane 2015 |
| SCIg non-inferior to IVIg for maintenance | Class I, Level A | van Schaik et al. Lancet Neurol 2018 (PATH Trial) |
| EFNS/PNS diagnostic criteria for CIDP | Expert Consensus | Van den Bergh et al. J Peripher Nerv Syst 2021 |
| Rituximab for anti-MAG neuropathy | Class II, Level B | Dalakas et al. Ann Neurol 2009 |
| Rituximab for refractory CIDP | Class III, Level C | Benedetti et al. Neurology 2011 |
| Nodal/paranodal antibodies predict IVIg response | Class II, Level B | Querol et al. Neurology 2017 |
| Azathioprine as steroid-sparing agent | Class III, Level C | Hughes et al. Cochrane 2017 |
| Mycophenolate for CIDP | Class III, Level C | Gorson et al. Neurology 2004 |
| MRI nerve root enhancement supportive criterion | Class II, Level B | Van den Bergh et al. J Peripher Nerv Syst 2021 |
| INCAT scale for disability measurement | Expert Consensus | Merkies et al. Neurology 2002 |
| Treatment-related fluctuations in CIDP | Class II, Level B | Kuitwaard et al. J Peripher Nerv Syst 2009 |
CHANGE LOG¶
v1.0 (January 27, 2026) - Initial template creation - Comprehensive diagnostic workup including EFNS/PNS criteria - First-line treatments: IVIg (loading and maintenance), corticosteroids, PLEX - Subcutaneous immunoglobulin (SCIg) for home maintenance therapy - Second-line agents: azathioprine, mycophenolate, rituximab, cyclophosphamide - Special populations: anti-MAG positive, nodal antibody positive CIDP - CIDP variants coverage (typical, MADSAM, DADS, sensory, motor) - Structured dosing format for all medications - Functional monitoring scales (INCAT, ONLS, grip strength) - Comprehensive differential diagnosis
APPENDIX A: EFNS/PNS 2021 Diagnostic Criteria for CIDP¶
Clinical Criteria¶
Typical CIDP: - Progressive or relapsing symmetric proximal AND distal weakness - Sensory dysfunction of all four limbs - Developing over ≥8 weeks - Reduced or absent tendon reflexes in all limbs
Atypical CIDP (at least one of): - DADS: Predominantly distal weakness and sensory loss - MADSAM: Asymmetric weakness (Lewis-Sumner syndrome) - Focal: Single limb involvement - Motor: Predominantly motor without sensory involvement - Sensory: Predominantly sensory without motor involvement
Electrodiagnostic Criteria (at least 1 nerve meeting criteria)¶
Motor Nerve Criteria (must meet in ≥2 nerves for definite CIDP):
| Parameter | Criterion |
|---|---|
| Distal motor latency | ≥50% above ULN |
| Motor conduction velocity | ≤70% below LLN |
| F-wave latency | ≥30% above ULN |
| Conduction block | ≥50% amplitude reduction proximal vs distal |
| Temporal dispersion | ≥30% increase in duration proximal vs distal |
Supportive Criteria¶
- CSF: Elevated protein with WBC <10/mm³
- MRI: Enhancement or hypertrophy of nerve roots, brachial or lumbosacral plexus
- Objective clinical improvement with immunomodulatory treatment
- Nerve ultrasound: Increased cross-sectional area of peripheral nerves
- Nerve biopsy: Evidence of demyelination/remyelination
Diagnostic Categories¶
| Category | Criteria |
|---|---|
| Definite CIDP | Clinical criteria + ≥2 nerves meeting electrodiagnostic criteria |
| Probable CIDP | Clinical criteria + 1 nerve meeting electrodiagnostic criteria |
| Possible CIDP | Clinical criteria but electrodiagnostic criteria not fully met; supportive criteria present |
APPENDIX B: INCAT Disability Scale¶
Arm Disability Score: | Grade | Description | |-------|-------------| | 0 | No upper limb problems | | 1 | Symptoms not affecting function | | 2 | Symptoms affecting but not preventing function | | 3 | Symptoms preventing function but able to do some self-care | | 4 | Symptoms preventing function and most self-care | | 5 | Cannot use either arm for any purposeful movement |
Leg Disability Score: | Grade | Description | |-------|-------------| | 0 | Walking not affected | | 1 | Walking affected but can walk independently outdoors | | 2 | Uses unilateral support outdoors (cane, crutch, arm) | | 3 | Uses bilateral support outdoors (two canes, crutches, walker, arm) | | 4 | Uses wheelchair outdoors; walks with support indoors | | 5 | Restricted to wheelchair, unable to stand/walk |
Total Score: Sum of arm + leg scores (0-10)
APPENDIX C: IVIg to SCIg Conversion Guide¶
Conversion Formula¶
Weekly SCIg dose = Monthly IVIg dose ÷ 4
Example Conversions¶
| Monthly IVIg Dose | Weekly SCIg Dose | Notes |
|---|---|---|
| 40 g (0.5 g/kg for 80 kg) | 10 g/week | Can divide into 2-3 infusions/week |
| 60 g (0.75 g/kg for 80 kg) | 15 g/week | Can divide into 3-4 infusions/week |
| 80 g (1 g/kg for 80 kg) | 20 g/week | May need 4-7 infusions/week |
SCIg Administration¶
Products: - Hizentra (20% concentration): Up to 25 mL per site - Gamunex-C (10% concentration): Up to 30 mL per site
Infusion Sites: - Abdomen, thighs, upper arms, hips - Rotate sites; use 2-4 sites per infusion
Advantages of SCIg: - Home administration - Stable IgG levels (no peaks/troughs) - Fewer systemic side effects - Greater independence
Training Required: - Initial training by infusion nurse (typically 2-3 sessions) - Return demonstration of sterile technique - Recognition of adverse reactions
APPENDIX D: Monitoring Timeline¶
First Year of Treatment¶
| Time | Monitoring |
|---|---|
| Baseline | NCS/EMG, MRI (if not done), labs (CBC, CMP, HbA1c, SPEP/UPEP, immunoglobulins), INCAT score, grip strength |
| 2 weeks | If on IVIg: renal function |
| 4-6 weeks | Clinical assessment; titrate steroids |
| 3 months | Clinical exam, labs (CBC, CMP, LFTs if on immunosuppressants), HbA1c if on steroids |
| 6 months | Clinical exam, repeat NCS/EMG, INCAT score, labs |
| 12 months | Comprehensive review: NCS/EMG, DEXA (if on steroids), INCAT, consider dose reduction trial |
Maintenance Phase (Annual)¶
- Clinical exam with INCAT/ONLS q6-12 months
- NCS/EMG if clinical change
- Labs: CBC, CMP, LFTs q3-6 months (based on therapy)
- DEXA q1-2 years if on steroids
- HbA1c q3-6 months if on steroids or diabetic
- Immunoglobulin levels q6-12 months if on IVIg/SCIg
Dose Reduction Trial¶
Consider after 6-12 months of stable disease: 1. Reduce IVIg by 10-20% or extend interval by 1 week 2. Monitor closely over 4-8 weeks 3. If stable, continue slow reduction 4. If worsening, return to prior effective dose 5. Some patients require lifelong therapy; others may achieve drug-free remission