From the Department of Neurology (L.A., A.-M.B., C.S., K.D.), University Hospital of Würzburg; Neuroimmunology Section (A.H., K.-P.W., R.J., F.L.), Institute of Clinical Chemistry, University Hospital of Schleswig-Holstein Campus Kiel; Department of Neurology (P.K.), University Hospital of Magdeburg; and Institute for Clinical Neurobiology (C.V.), University Hospital of Würzburg, Germany.
Neurol Neuroimmunol Neuroinflamm. 2020 Jul 24;7(5). doi: 10.1212/NXI.0000000000000817. Print 2020 Sep.
To determine whether IgG subclasses of antiparanodal autoantibodies are related to disease course and treatment response in acute- to subacute-onset neuropathies, we retrospectively screened 161 baseline serum/CSF samples and 66 follow-up serum/CSF samples.
We used ELISA and immunofluorescence assays to detect antiparanodal IgG and their subclasses and titers in serum/CSF of patients with Guillain-Barré syndrome (GBS), recurrent GBS (R-GBS), Miller-Fisher syndrome, and acute- to subacute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). We evaluated clinical data retrospectively.
We detected antiparanodal autoantibodies with a prevalence of 4.3% (7/161), more often in A-CIDP (4/23, 17.4%) compared with GBS (3/114, 2.6%). Longitudinal subclass analysis in the patients with GBS revealed IgG2/3 autoantibodies against Caspr-1 and against anti-contactin-1/Caspr-1, which disappeared at remission. At disease onset, patients with A-CIDP had IgG2/3 anti-Caspr-1 and anti-contactin-1/Caspr-1 or IgG4 anti-contactin-1 antibodies, IgG3 being associated with good response to IV immunoglobulins (IVIg). In the chronic phase of disease, IgG subclass of one patient with A-CIDP switched from IgG3 to IgG4.
Our data (1) confirm and extend previous observations that antiparanodal IgG2/3 but not IgG4 antibodies can occur in acute-onset neuropathies manifesting as monophasic GBS, (2) suggest association of IgG3 to a favorable response to IVIg, and (3) lend support to the hypothesis that in some patients, an IgG subclass switch from IgG3 to IgG4 may be the correlate of a secondary progressive or relapsing course following a GBS-like onset.
为了确定抗神经节段 IgG 亚类与急性至亚急性发作性神经病的病程和治疗反应是否相关，我们回顾性筛选了 161 份基线血清/CSF 样本和 66 份随访血清/CSF 样本。
我们使用 ELISA 和免疫荧光测定法检测了格林-巴利综合征（GBS）、复发性 GBS（R-GBS）、米勒-费舍尔综合征和急性至亚急性发作性慢性炎症性脱髓鞘性多发性神经病（A-CIDP）患者血清/CSF 中的抗神经节段 IgG 及其亚类和滴度。我们回顾性评估了临床数据。
我们检测到抗神经节段自身抗体的患病率为 4.3%（7/161），在 A-CIDP（4/23，17.4%）中比 GBS（3/114，2.6%）更常见。GBS 患者的纵向亚类分析显示，针对 Caspr-1 和针对抗接触蛋白-1/Caspr-1 的 IgG2/3 自身抗体消失在缓解期。在疾病发作时，A-CIDP 患者具有 IgG2/3 抗 Caspr-1 和抗接触蛋白-1/Caspr-1 或 IgG4 抗接触蛋白-1 抗体，IgG3 与 IV 免疫球蛋白（IVIg）的良好反应相关。在疾病的慢性期，一名 A-CIDP 患者的 IgG 亚类从 IgG3 转变为 IgG4。
我们的数据（1）证实并扩展了先前的观察结果，即抗神经节段 IgG2/3 但不是 IgG4 抗体可发生在表现为单相 GBS 的急性发作性神经病中，（2）表明 IgG3 与 IVIg 的良好反应相关，（3）支持这样的假设，即在某些患者中，从 IgG3 到 IgG4 的 IgG 亚类转换可能是类似于 GBS 发作后的继发性进行性或复发性病程的相关因素。
