Chronic inflammatory polyradiculoneuropathy cidp
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Chronic Inflammatory Polyradiculoneuropathy (CIDP). Outline. General Clinical Manifestations Diagnosis Treatment and prognosis. CIDP. Acquired, immune-mediated polyradiculoneuropathy

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Outline

  • General

  • Clinical Manifestations

  • Diagnosis

  • Treatment and prognosis


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CIDP

  • Acquired, immune-mediated polyradiculoneuropathy

  • Heterogeneous disorder with a wide range of clinical expression ranging from subacute to a progressive or relapsing-remitting course

  • Diagnosis is based on clinical symptoms and signs, electrodiagnostic studies, CSF examination, and other laboratory tests


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CIDP: Clinical Manifestations

  • Demyelination

    • May be detected on nerve conduction studies or nerve biopsy

    • Multifocal demyelination is a diagnostic hallmark of CIDP, but distribution of demyelinative lesions varies among patients

  • Weakness

    • Characteristically, involves both proximal and distal muscles

    • Typically symmetric, but can begin asymmetrically

  • Sensory symptoms are common but motor symptoms usually predominate

  • Autonomic system dysfunction can occur


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CIDP: Clinical Manifestations

  • Slow progressive course is seen in approximately 2/3 of cases

  • Children usually have a more precipitous onset of symptoms

  • Relapsing course with partial or complete recovery between recurrences is seen in approximately 1/3 of cases

    • Periods of worsening and improvement usually last weeks or months

    • Patients with a younger age of onset are said to have a higher frequency of relapsing course


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CIDP: Incidence

  • In one study, prevalence was estimated to range from 1-7.7 per 100,000

    • These are likely underestimates, since the criteria to select cases were strict

  • CIDP accounted for 13% of patients seen in one neuromuscular center

  • Incidence increases with increasing age

    • Children are rarely affected


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CIDP: Pathophysiology

  • In one study, CIDP occurred within a few weeks after an infectious event in 16% of the patients

    • Because of the insidious onset, documenting precipitating illnesses or events is very difficult

    • Both respiratory and gastrointestinal infections have been cited, but no causative organism has been identified

    • With Guillain-Barre syndome, the most common preceding infection is Campylobacter

Bouchard et al. NEUROLOGY 1999;52:498–503


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CIDP: Regional Variants

  • The classical phenotype that suggests CIDP is the presence of proximal and distal weakness, with large fiber sensory loss and areflexia

    • Few patients present with classic symtpoms

  • Some authors have suggested subclassifications based on the clinical phenotype in order to aid in diagnosis and treatment

    • Currently these subclasses are not thought to be distinct diseases


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Lewis-Sumner syndrome

Distal acquired demyelinating sensory neuropathy and sensory CIDP

Multifocal demyelinating neuropathy with persistent conduction block

Distal CIDP

Sensory CIDP

ANTI-MAG (Myelin Associated Glycoprotein) Neuropathy

CIDP with Hypertrophic nerves

Subacute demyelinating polyneuropathy

Chronic Inflammatory demyelinating neuropathies

CIDP: Regional Variants


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CIDP: Associated Conditions

  • Most frequently CIDP is an idiopathic illness

  • Has been known to occur with several conditions

    • In these cases, the associated condition is included in the main diagnosis to separate those cases from the idiopathic variety

      • Example: “CIDP with HIV infection”


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CIDP: Associated Conditions

  • HIV infection

    • Mild lymphocytic pleocytosis and increased gamma globulin level in the CSF are seen frequently

  • Hodgkin lymphoma

    • Associated neuropathy is not caused by direct infiltration of the peripheral nerves but is a consequence of the autoimmune cascade that occurs with this disease, but the mechanism is not completely clear

  • Paraproteinemias and/or plasma cell dyscrasias


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    CIDP: Associated Conditions

    • CIDP is seen with monoclonal gammopathies (eg MGUS), most frequently gammopathy of immunoglobulin M (IgM)

      • Evidence suggests that CIDP with IgM MGUS has specific clinical and electrophysiologic characteristics

        • Usually predominance of distal weakness with sensory symptoms greater than motor

    • Multiple sclerosis

      • Reports describe CNS white matter changes in patients with CIDP

      • Whether a true association exists between CIDP and multiple sclerosis remains unclear


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    CIDP: Associated Conditions

    • Systemic lupus erythematosus

    • Chronic active hepatitis (B or C)

      • CIDP associated with hepatitis should be differentiated from cryoglobulinemic vasculitis

      • The latter causes either symmetric distal sensorimotor polyneuropathy or mononeuropathy multiplex but on pathologic examination shows wallerian degeneration and not the segmental demyelination seen in CIDP


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    CIDP: Associated Conditions

    • Inflammatory bowel disease

      • CIDP has been described in association with Crohn disease and other inflammatory bowel conditions, although no direct correlation between the two afflictions is known

      • The mechanism of development of CIDP is presumed to be an autoimmune abnormality that is also causing the primary problem in inflammatory bowel disease, although the details are not known


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    CIDP: Associated Conditions

    • Diabetes mellitus

      • Increasing evidence supports the suggestion that some patients with diabetes who have severe neuropathy or unusually progressive neuropathy may have CIDP superimposed on their diabetic disorder

      • Diabetes may predispose patients to CIDP

    • Pregnancy

      • Known to worsen CIDP

      • Worsening usually occurs in the third trimester or in the postpartum period


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    CIDP: Monitoring and Prognosis

    • Sometimes difficult to assess the activity of a chronic neuropathy

    • Nerve biopsy can be used to detect active nerve lesions and inflammatory infiltrates

    • Axonal loss has more long-term prognostic impact than active demyelination or inflammatory infiltrates in demyelinating disorders of the peripheral and central nervous systems


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    CIDP: Diagnosis

    • The diagnosis of CIDP is typically based on the clinical presentation, absence of other causes of the neuropathic syndrome, and results of electrodiagnostic studies

    • Presence of increased cerebrospinal fluid (CSF) protein and demyelinating changes on nerve biopsy are supportive of the diagnosis, but these are not always present


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    CIDP: Diagnosis

    • Because of the clinical heterogeneity and the lack of a diagnostic test, various diagnostic criteria have been proposed

      • In one series of patients all of whom had proximal and distal weakness and in whom 95% of patients had improvement with treatment, only 30% had the classic triad of slow nerve conduction velocity, elevated CSF protein and demyelination on nerve biopsy


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    American Association of Neurology: Criteria

    • Developed criteria for the identification of patients with CIDP for research studies

      • Pathologic criteria

      • Electrophysiologic criteria: Require 3 demyelinating range abnormalities (either slow conduction velocity, prolonged distal motor latencies or F wave latencies or conduction block) in 2 nerves

      • Criteria are not sensitive and may miss more than 50 % of patients with CIDP

      • Specificity approaches 100%

      • Majority of patients seen in clinical practice fail to meet all of the criteria


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    Laboratory Studies: CSF

    • Protein level is increased significantly in 80% of patients

      • Usually between 50 and 200 mg/dL, but can be higher

    • 10% of patients also have mild lymphocytic pleocytosis (<50 cells) and increased gamma globulin (usually associated with HIV infection)

    • CBC, sedimentation rate, antinuclear antibody, biochemistry profile, and serum and urine immunoelectrophoresis are necessary to exclude important associated systemic disorders


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    CIDP: EMG

    • Critical test to determine whether the disorder is truly a peripheral neuropathy and whether the neuropathy is demyelinating

    • Findings of a demyelinating neuropathy

      • Multifocal conduction block or temporal dispersion of compound muscle action potential

      • Prolonged distal latencies

      • Variable conduction slowing to less than 70% of normal

      • Absent or prolonged F wave latencies

      • As the disease progresses, patients tend to develop secondary axonal degeneration


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    CIDP: Peripheral nerve biopsy

    • Indications

      • Patients in whom the diagnosis is not completely clear

      • Cases where other causes of neuropathy (eg, hereditary, vasculitic) cannot be excluded

      • Caes where profound axonal involvement is observed on EMG

      • Some experts recommend biopsy for most patients prior to initiating immunosuppressive therapy


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    CIDP: Histologic Findings

    • Interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages with local edema

    • Evidence exists of segmental demyelination and remyelination with occasional onion bulb formation, particularly in relapsing cases

    • Some evidence of axonal damage also is observed, with loss of myelinated nerve fibers

    • The inflammatory infiltrate with neutrophil infiltration is observed in only a minority of patients


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    CIDP: Histology

    • Note the decreased density of nerve fibers (arrows)

    • Demyelinated fibers (D)

    • Fibers undergoing active macrophagemediated demyelination (M)

    Bouchard, et al. NEUROLOGY 1999;52:498–503


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    CIDP: Therapy

    • Prednisone, IVIg and plasmapheresis have all been demonstrated to be effective in controlled clinical trials

      • In one study, response was seen to at least 1 of these 3 main therapies in 66% of patients

    • Only 1/3 of patients have a sustained remission after initial treatment and most require ongoing treatment

    • Early treatment is advisable to prevent axonal loss and motor neuron loss which leads to functional decline

      • May be irreversible


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    CIDP: Therapy

    • A positive therapeutic response is measured by improvement or stabilization of previously documented progressive weakness, sensory loss, or ataxia

    • In responsive patients, treatment is continued until maximal improvement or stabilization is achieved, at which point it can be tapered or discontinued

    • If there is further deterioration or a relapse, the therapy can be re-instituted

    • Patients with chronic progressive disease require maintenance therapy, although tapering the treatment can be re-attempted periodically to determine continued need


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    CIDP: Therapy

    • Prednisone

      • First line therapy

    • Agents used for refractory patients

      • Cyclosporine (Sandimmune, Neoral)

      • Cyclophosphamide (Cytoxan)

      • Azathioprine (Imuran)

      • Mycophenolate (CellCept)


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    CIDP: Therapy

    • Neuropathic pain

      • Antiepileptics

        • Carbamazepine (Tegretol)

        • Gabapentin (Neurontin)

      • Tricyclic antidepressants

        • Amitriptyline (Elavil)


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    CIDP: Plasmapheresis

    • Several controlled studies confirmed benefit

    • Proposed mechanism

      • Removal of antibodies and complement components that are responsible for immune-mediated damage of peripheral nerves

    • Has been shown to have similar efficacy as IVIg in treatment of CIDP


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    CIDP: Plasmapheresis

    • Treatment regimens

      • Not standardized due to a lack of controlled studies

      • Common regimen

        • 3 plasma exchanges per week for first 2 weeks

        • Additional treatment is determined by clinical response


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    IV Ig

    • Solution composed mostly of heterogenous human IgG but also small amounts of IgA and IgM

    • Proposed mechanism of action

      • IVIg contains random set of antibodies that would neutralize immune factors, causing damage to peripheral nerve in CIDP

      • Activates complement cascade and provides multitude of antibodies capable of neutralization of many microorganisms, toxins, viruses, and presumably autoantibodies


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    IV Ig

    • Used in infectious diseases to provide immediate passive immunity in situations in which time constraints do not allow development of active immunity via vaccination

    • Also used to treat multiple immune-mediated conditions, such as idiopathic thrombocytopenic purpura, GBS, and myasthenia gravis


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    CIDP: IV Ig

    • Several studies showed significant benefit in CIDP

      • Useful alternative to plasmapheresis

    • On average, improvement seen by day 10 and continues through day 42

    • Serum half-life is approximately 21-29 days

    • Patients usually require repeated treatments every few weeks or months to maintain remission or treat recurrences


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    CIDP: Prognosis

    • The outcome of CIDP is difficult to predict owing to the variety of clinical patterns and evolution

    • If left untreated, it can become disabling, with loss of ability to ambulate, work, or function independently


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    References

    • “Acquired demyelinating neuropathy”. Brain. 119. 257–270.

    • Ad Hoc Subcommittee of the American Academy of Neurology, AIDS Task Force (1991). “Research criteria for diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)”. Neurology. 41. 617–618.


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    References

    • Bouchard, et al. “Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy”.Neurology. February (1 of 1). 1999. 52. 498-503.

    • Kuwabara, S, et al. “Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy”. Journal of Neurology Neurosurgery and Psychiatry. 2002. 72. 37-42.

    • Latov N. “Diagnosis of CIDP”. Neurology. 59. S2–S6.


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