Educational draft · awaiting clinical reviewThe full textbook explains uncertainty but does not replace live national or local guidance, specialist advice, or current prescribing information.
Identify chronic or relapsing immune demyelinating neuropathy, require electroclinical concordance before long-term treatment, and monitor objective response to IVIg, corticosteroids or plasma exchange.
CIDP is an acquired immune-mediated neuropathy affecting myelin, nodes and sometimes axons. Typical disease develops over more than 8 weeks with symmetric weakness involving shoulder or hip as well as distal muscles, sensory impairment in more than one limb and generalized hyporeflexia or areflexia. Difficulty rising, climbing, lifting and using hands occurs alongside numbness and sensory ataxia. Variants include distal, multifocal or Lewis-Sumner, focal, motor and sensory-predominant syndromes, but diagnostic certainty is lower and mimics more numerous. Cranial involvement can occur; prominent sphincter dysfunction, a sensory level, severe pain or major autonomic failure should prompt alternative localisation.
Electrodiagnosis anchors the label. Multiple motor nerves must show acquired demyelinating abnormalities meeting EAN/PNS or locally adopted criteria, with temperature, entrapment and axonal loss interpreted carefully. Diabetes can cause modest slowing but rarely the convincing multifocal conduction block of acquired demyelination. CSF protein elevation with low cells supports proximal root involvement, yet high protein alone is a common source of overdiagnosis. MRI or ultrasound nerve enlargement, sensory studies, treatment response and, in selected cases, nodal antibodies provide supportive evidence. Screen blood count, glucose, renal, liver, thyroid, B12, immunoglobulins and electrophoresis or immunofixation and history-led infection, autoimmune, amyloid and genetic causes. Nerve biopsy is rarely needed.
Most CIDP reflects acquired immune attack on peripheral myelin or nodal structures without a single identified trigger, producing chronic or relapsing sensorimotor dysfunction.
Monoclonal proteins and antibodies to nodal or paranodal targets define clinically important subgroups with different phenotypes, treatment responses and associated disease.
Diabetes, immune disease, infection and malignancy may coexist or occasionally contribute, but their presence does not replace the need for electroclinical evidence of acquired demyelination.
Antibodies, macrophages and lymphocytes damage Schwann-cell myelin and nodal organisation across multiple peripheral nerves and roots.
Demyelinated segments conduct slowly or fail, causing temporal dispersion, weakness, sensory loss and reduced reflexes across proximal and distal muscles.
Repeated inflammatory attacks and prolonged conduction failure injure axons, converting treatable dysfunction into fixed weakness, wasting and sensory disability.
More than 8 weeks of symmetric proximal and distal weakness, sensory loss and generalized areflexia is the core clinical phenotype.
Objective deterioration and improvement separated over months can occur, but day-to-day fatigue or pain fluctuation should not be labelled immune relapse without examination.
An apparent GBS episode that continues progressing beyond 8 weeks or has repeated treatment-related deteriorations may evolve into a chronic immune neuropathy.
Asymmetric sensory and motor deficits in individual nerve distributions with demyelinating physiology suggests multifocal acquired demyelinating sensory and motor neuropathy.
Weakness with little sensory loss can represent motor CIDP, but motor neurone disease and multifocal motor neuropathy require careful exclusion.
A monophasic illness reaching its worst within weeks favours GBS; progression or relapse beyond the expected acute course raises CIDP or acute-onset CIDP.
Uniform slowing, longstanding cavus feet and a family history favour inherited neuropathy, while conduction block and relapsing proximal weakness support CIDP.
Predominantly axonal length-dependent loss, prominent autonomic features or a specific protein pattern may indicate another neuropathy that will not respond like CIDP.
Upper motor-neurone signs, a sensory level or preserved sensory studies should redirect localisation away from a diffuse demyelinating sensorimotor neuropathy.
Monoclonal protein, severe pain, weight loss, autonomic failure, family foot deformity or poor objective treatment response raises amyloid, POEMS, anti-MAG, hereditary or other disease.
Proximal and distal motor failure impairs rising, stairs, grip and foot clearance, causing falls and loss of independence.
Large-fibre loss destabilises gait and removes protective sensation, while neuropathic pain and tremor may persist despite improved strength.
Delayed recognition or repeated relapse causes axonal loss that limits recovery even when inflammation is later suppressed.
Immunoglobulin, corticosteroids and plasma exchange carry thrombotic, metabolic, infective, vascular-access and resource burdens, making objective response monitoring essential.
Mild CSF protein elevation is frequent in older adults, diabetes and spinal stenosis and cannot replace acquired demyelination on expert neurophysiology.
Feeling energised after an infusion is real but non-specific; strength, walking and disability measures determine whether immune nerve function changed.
Stopping immune demyelination may stabilise disease without restoring longstanding denervated muscle, so incomplete recovery is not always treatment failure.
Corticosteroids can worsen motor-predominant disease, making accurate sensory history and electrophysiological classification important before first-line selection.
Repeated matched measurements before and after infusions distinguish biological end-of-dose weakness from pain, fatigue or expectation and guide interval adjustment.
Diagnosing CIDP from raised CSF protein and diabetic neuropathy without neurophysiological evidence meeting acquired demyelination criteria.
Calling mild slowing in cold limbs or across common entrapment sites diffuse inflammatory demyelination.
Using subjective fatigue improvement as the sole reason for lifelong immunoglobulin without objective baseline and withdrawal testing.
Starting corticosteroids reflexively in pure motor CIDP despite potential deterioration and the preference for IVIg.
Missing paraprotein, amyloid, POEMS or hereditary neuropathy when systemic features and poor treatment response contradict typical CIDP.
Escalating immune therapy indefinitely when severe secondary axonal loss, not ongoing demyelination, limits functional recovery.
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A patient develops symmetrical proximal and distal weakness, sensory loss and absent reflexes that progresses steadily for 12 weeks. Nerve conduction meets acquired demyelination criteria. Which diagnosis best fits?
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.
Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes
IVIg, corticosteroids and plasma exchange are evidence-based initial therapies selected around phenotype, speed, comorbidity, access and preference. A common IVIg induction is total 2 g/kg over 2–5 days, followed by maintenance tailored to objective wear-off and function. Plasma exchange acts quickly but requires vascular access and ongoing sessions. Corticosteroid regimens may be daily oral or pulsed through specialist practice, but diabetes, osteoporosis, infection, psychiatric effects and motor-CIDP deterioration influence choice. Subcutaneous immunoglobulin is an option for maintenance after IVIg response, not a rapid induction for major deterioration. If first-line treatments fail despite secure diagnosis, specialist immunosuppressive options may be considered with limited evidence. Rehabilitation, falls management and pain care continue while immune treatment prevents further injury; longstanding axonal loss may cap recovery.
Thrombosis, renal injury, haemolysis, aseptic meningitis, headache, fluid load and reaction risk require product-specific monitoring and periodic need review.
Diabetes, hypertension, osteoporosis, infection, mood, eye and adrenal effects are substantial; pure motor CIDP can deteriorate and generally starts with IVIg.
Local reactions are common; systemic reactions and thrombosis remain possible, training is required and it is not the usual rescue for acute major decline.