October 28, 2021

Autoantibody testing in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic immune-mediated inflammatory polyneuropathy. It is progressive or relapsing for at least two months, results in peripheral nerve demyelination, and responds to immunosuppressive therapies.

CIPS can be classified as either “typical” or “variants.” Typical CIDP is the most common form and is characterized by symmetric proximal and distal muscle weakness.

October 28, 2021

Autoantibody testing in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic immune-mediated inflammatory polyneuropathy. It is progressive or relapsing for at least two months, results in peripheral nerve demyelination, and responds to immunosuppressive therapies.

CIPS can be classified as either “typical” or “variants.” Typical CIDP is the most common form and is characterized by symmetric proximal and distal muscle weakness.

There are great challenges in diagnosing CIDP, not least from the fact that over half of patients have alternative diagnoses with features consistent with CIDP. Improvements to the diagnosis can be made by analyzing patients for the presence of specific autoantibodies.

Patients with antibodies against nodal-paranodal cell-adhesion molecules Contactin-1 (CNTN1), Neurofascin-155 (NF155), or Contactin-associated protein 1 (Caspr1) often have specific clinical characteristics yet show poor response to conventional immunotherapy.

  • Anti-CNTN1 antibodies are present in 2-9% of patients with CIDP. These patients have rapid and aggressive onset of the symptoms, predominant involvement of motor fibers, and axonal damage.
  • Anti-NF155 antibodies are present in 4-18% of patients with CIDP. Patients are typically young at onset and have a subacute or chronic disease course, distal weakness, ataxia, and tremor.
  • Anti-Caspr1 antibodies present as an acute/subacute neuropathy frequently associated with ataxia, neuropathic pain, and cranial nerve involvement.

How we can help

Wieslab Diagnostic Services can help in the diagnosis of CIDP. We offer a CIDP panel as well as individual tests for:

IgG autoantibodies against NF155 and CNTN1 have been identified in a subgroup of patients with CIDP that often have a poor response to standard treatment. Since these treatment-resistant cases can be responsive to rituximab, testing for IgG-autoantibodies can be a valuable tool in personalizing treatment for this group.