History and exam

Key diagnostic factors

common

disease progression

Most patients have either a chronic progressive course or a relapsing-remitting course over >8 weeks.[3]​​​[5][6]​​

Rarely, patients present with subacute onset over 4-8 weeks with a monophasic course.[5][7][8]​​​ This presentation is more common in children.[5][9]

weakness

Over 90% of patients report weakness that is typically symmetrical, involves all four limbs, and affects proximal and distal muscles.[3][4]​​[5][6]​​

Rarely, weakness affects arms more than legs or is asymmetrical.​[12][14][67]

Pure motor syndromes occur rarely.[1][68]​​[69]​​​

Distal CIDP (also known as distal acquired demyelinating symmetrical neuropathy) presents with predominant distal weakness.[1][10]​​

altered sensation

Numbness or paraesthesia is noted in about 75% of patients.[3][4]​​[5][6]​​ The prevalence of pain due to CIDP at any time during the disease course was estimated as 46% in one systematic review.[37]

Nearly all patients have symmetrical distal sensory loss to large- and small-fibre modalities.​[3][4]​​[5][6]​​

Rarely, patients present with only sensory symptoms, typically distal paraesthesias and dysaesthesias.[1][38][39]

Pure sensory syndromes occur in <10% of patients and may be either mixed large- and small-fibre or predominantly large-fibre, in which sensory ataxia is the major manifestation.[38][39][70][71]​​​ In pure sensory syndromes, nerve conduction studies may show only mild abnormalities not meeting typical electrodiagnostic criteria. Elevated cerebrospinal fluid protein, abnormal somatosensory evoked potentials, and demyelination on nerve biopsy may be present.[38][39]

decreased deep tendon reflexes

Hyporeflexia or areflexia is a required component in most sets of diagnostic criteria and occurs in >90% of patients.[3][4]​​[5][6]​​

Other diagnostic factors

common

incoordination

Gait difficulty is usually due to weakness. However, presentation with a sensory ataxia is also described rarely in patients.[70][71]

age 40 to 60 years

Can occur at any age (although rare in childhood), but the most common age of onset is between 40 and 60 years.[1][5]

uncommon

preceding infection

Because of the chronic nature of the disease, it can be difficult to document a temporal relationship, but most studies suggest that 10% to 30% of patients have a preceding infection.​[4][6][17]

absence of exposure to neuropathy-causing drugs

Drug or toxin exposure that is likely to have caused the neuropathy (e.g., diphtheria, buckthorn, amiodarone, tacrolimus) may rule out a diagnosis of CIDP.[32][33]

dyspnoea

Shortness of breath requiring intubation occurs in <5% of patients.[4]​​[70][72]

facial weakness

Occurs in about 15% of patients.​[4]​​[5][6]

dysarthria

Occurs in about 15% of patients.​[4]​​[5][6]

dysphagia

Occurs in about 15% of patients.​[4]​​[5][6]

urinary incontinence

Occurs in about 25% of patients and is often mild.​[73][74]

urinary urgency or hesitancy

Occurs in about 25% of patients and is often mild.​[73][74]

impotence

Occurs in about 25% of patients.​[73][74]

orthostatic hypotension

Occurs in about 25% of patients.​[73][74]

papilloedema

May be present but is usually mild.[75][76][77]

vision loss

May be present but is usually mild.[75][76][77]

spasticity

May be present but is usually mild.[75][76][77]

Risk factors

weak

male sex

CIDP is more common in men than in women, with reported male-to-female ratios of between 1.4 and 4.4.[15]

autoimmune diseases

May co-exist with other autoimmune diseases, such as systemic lupus erythematosus, connective tissue disease, inflammatory bowel disease, thyroid disease, sarcoidosis, glomerulonephritis, chronic active hepatitis, or graft-versus-host disease.[19][20]

diabetes mellitus

There is an increased incidence of CIDP in patients with diabetes mellitus, and severity of CIDP may be worse in these patients.[18][19][20]​​​

infection

Can occur in association with infection, including HIV.[19][20]

monoclonal gammopathy of undetermined significance (MGUS)

About 10% to 15% of patients have a benign MGUS, most often with an IgA or IgG paraprotein; MGUS may affect disease course and response to treatment.[2][19][20]​​​

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