Terminal complement activation is increased and associated with disease severity in CIDP

Area of Science:

• Neurology
• Immunology
• Pathogenesis of Neurological Disorders

Background:

• Chronic inflammatory demyelinating polyneuropathy (CIDP) is a prevalent autoimmune neuropathy.
• Both cellular and humoral immune responses are implicated in CIDP pathogenesis.
• The efficacy of plasmapheresis suggests a circulating factor contributes to nerve damage.

Purpose of the Study:

• To investigate the role of complement system activation in treatment-naïve CIDP patients.
• To correlate complement activation markers with clinical disease severity in CIDP.

Main Methods:

• Measurement of serum and cerebrospinal fluid (CSF) levels of C5a and soluble terminal complement complex (sTCC) in CIDP patients.
• Assessment of clinical disease severity using the Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale.

Main Results:

• Treatment-naïve CIDP patients exhibited elevated serum and CSF levels of C5a and sTCC.
• Systemic complement activation significantly correlated with clinical disease severity (INCAT scale).

Conclusions:

• Complement activation, specifically involving C5a and sTCC, plays a role in peripheral nerve injury in CIDP.
• These findings suggest that complement inhibition could be a potential therapeutic avenue for managing CIDP.