Department of Neuroinflammation Institute of Experimental Immunology University of Zürich Zürich Switzerland.
Department of Rheumatology and Clinical Immunology Charité-University Medicine Berlin Berlin Germany.
Ann Clin Transl Neurol. 2016 Jul 25;3(9):730-5. doi: 10.1002/acn3.331. eCollection 2016 Sep.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. While both cell-mediated and humoral mechanisms contribute to its pathogenesis, the rapid clinical response to plasmapheresis implicates a circulating factor responsible for peripheral nerve injury. We report that treatment-naïve patients with CIDP show increased serum and CSF levels of the anaphylatoxin C5a and the soluble terminal complement complex (sTCC). Systemic terminal complement activation correlates with clinical disease severity as determined by the Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale. These data indicate that complement activation contributes to peripheral nerve injury and suggest that complement inhibition should be explored for its potential therapeutic merit in CIDP.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是最常见的慢性自身免疫性神经病。虽然细胞介导和体液机制都有助于其发病机制，但血浆置换的快速临床反应表明存在导致周围神经损伤的循环因子。我们报告称，CIDP 的初治患者血清和 CSF 中的过敏毒素 C5a 和可溶性末端补体复合物（sTCC）水平升高。全身末端补体激活与炎症性神经病病因和治疗（INCAT）残疾量表确定的临床疾病严重程度相关。这些数据表明补体激活有助于周围神经损伤，并表明应探索补体抑制在 CIDP 中的潜在治疗价值。
