Etiology

There are no clearly defined genetic or environmental risk factors.[2]​ Infection, immunization, or surgical procedures precede CIDP in approximately 10% to 30% of patients.​[4][6][17]​​​​ 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]​​​​​ CIDP can also occur in association with infection, including HIV.​​​[19][20] About 10% to 15% of patients have a benign monoclonal gammopathy of undetermined significance (MGUS), most often with an IgA or IgG paraprotein; MGUS may affect disease course and response to treatment.​​​[2][19][20] CIDP may also coexist 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]

Pathophysiology

Evidence suggests that CIDP is an autoimmune disease directed against peripheral nerve myelin. Nerve biopsies from patients with CIDP suggest underlying cell-mediated immunity as a mechanism of disease. Endoneurial infiltrates consist mainly of CD4+ and CD8+ T cells and macrophages.[21][22]​​ Immunoglobulin and complement have been immunolocalized to myelin sheaths of affected nerves, and a variety of cytokines, chemokines, costimulatory molecules, adhesion molecules, growth factors, and transcription factors are upregulated in biopsies from patients with CIDP.​​​​[2]​ Humoral immunity also likely plays a role (especially given the discovery of disease mediated by antiparanodal and antinodal antibodies), but autoantibodies directed against myelin proteins and lipids, such as gangliosides, sulfatides, galactocerebroside, and myelin protein zero, have been found in only a minority of patients.​​​[2]

Classification

European Academy of Neurology/Peripheral Nerve Society (EAN/PNS)[1]

The 2021 EAN/PNS guideline defines two levels of diagnostic certainty for CIDP:[1]

  • CIDP

  • Possible CIDP.

The most common presentation is typical CIDP.[1][2]

CIDP variants are:[1]

  • Distal CIDP (also known as distal acquired demyelinating symmetric neuropathy)

  • Multifocal CIDP (also known as multifocal demyelinating neuropathy with persistent conduction block, Lewis-Sumner syndrome; multifocal acquired demyelinating sensory and motor neuropathy [MADSAM]; multifocal inflammatory demyelinating neuropathy)

  • Focal CIDP

  • Motor CIDP

  • Motor-predominant CIDP

  • Sensory CIDP

  • Sensory-predominant CIDP.

See Diagnostic criteria.

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