Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy.
Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy.
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DOI：
10.1002/acn3.248
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发表时间：
2015-10
影响因子：
5.3
通讯作者：
Kira J
中科院分区：
文献类型：
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作者：
Ogata H;Yamasaki R;Hiwatashi A;Oka N;Kawamura N;Matsuse D;Kuwahara M;Suzuki H;Kusunoki S;Fujimoto Y;Ikezoe K;Kishida H;Tanaka F;Matsushita T;Murai H;Kira J
目的探讨抗神经成束蛋白155（NF 155）抗体阳性的慢性炎性脱髓鞘性多发性神经病（CIDP）。使用稳定表达人NF 155或NF 186的HEK 293细胞系，通过流式细胞术测量来自我们诊所诊断的50名连续CIDP患者、32名多发性硬化症患者、40名其他神经病患者（包括26名格林-巴利综合征（GBS）/费舍尔综合征）和30名健康对照的血清中的抗NF抗体。从其他诊所转介的另外4名具有抗NF 155抗体的CIDP患者入组进行临床表征。CIDP患者抗NF 155抗体阳性率为18%（9/50），均以IgG 4亚类为主。除1例GBS患者携带IgG 1抗NF 155抗体外，其他受试者均为阳性。没有抗NF 155抗体携带者具有抗NF 186抗体。与抗NF 155抗体阴性患者相比，抗NF 155抗体阳性CIDP患者的发病年龄明显更小，垂足、步态障碍、震颤和远端获得性脱髓鞘对称表型的频率更高，磁共振成像神经造影显示颈根直径更大，脑脊液蛋白水平更高，远端和F波潜伏期更长。所有抗NF 155抗体阳性的CIDP患者神经造影检查均发现颈和腰骶神经根/神经丛明显对称性肥大。2例抗NF 155抗体患者的腓肠神经活检显示神经束膜下水肿和偶见结旁脱髓鞘，但无血管炎、炎性细胞浸润或洋葱球。在抗NF 155抗体阳性患者中，治疗应答者除了静脉注射免疫球蛋白外，每日口服皮质类固醇和/或免疫抑制剂的频率高于无应答者。抗NF 155抗体发生在CIDP患者的远端优势参与和对称性神经肥大的子集。
To investigate anti-neurofascin 155 (NF155) antibody-positive chronic inflammatory demyelinating polyneuropathy (CIDP). Sera from 50 consecutive CIDP patients diagnosed in our clinic, 32 patients with multiple sclerosis, 40 patients with other neuropathies including 26 with Guillain–Barré syndrome (GBS)/Fisher syndrome, and 30 healthy controls were measured for anti-NF antibodies by flow cytometry using HEK293 cell lines stably expressing human NF155 or NF186. Four additional CIDP patients with anti-NF155 antibodies referred from other clinics were enrolled for clinical characterization. The positivity rate for anti-NF155 antibodies in CIDP patients was 18% (9/50), who all showed a predominance of IgG4 subclass. No other subjects were positive, except one GBS patient harboring IgG1 anti-NF155 antibodies. No anti-NF155 antibody carriers had anti-NF186 antibodies. Anti-NF155 antibody-positive CIDP patients had a significantly younger onset age, higher frequency of drop foot, gait disturbance, tremor and distal acquired demyelinating symmetric phenotype, greater cervical root diameter on magnetic resonance imaging neurography, higher cerebrospinal fluid protein levels, and longer distal and F-wave latencies than anti-NF155 antibody-negative patients. Marked symmetric hypertrophy of cervical and lumbosacral roots/plexuses was present in all anti-NF155 antibody-positive CIDP patients examined by neurography. Biopsied sural nerves from two patients with anti-NF155 antibodies demonstrated subperineurial edema and occasional paranodal demyelination, but no vasculitis, inflammatory cell infiltrates, or onion bulbs. Among anti-NF155 antibody-positive patients, treatment responders more frequently had daily oral corticosteroids and/or immunosuppressants in addition to intravenous immunoglobulins than nonresponders did. Anti-NF155 antibodies occur in a subset of CIDP patients with distal-dominant involvement and symmetric nerve hypertrophy.