Autoimmune Aquaporin-4 Myopathy in Neuromyelitis Optica Spectrum
Autoimmune Aquaporin-4 Myopathy in Neuromyelitis Optica Spectrum
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DOI：
10.1001/jamaneurol.2014.775
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发表时间：
2014-08-01
期刊：
影响因子：
29
通讯作者：
Lucchinetti, Claudia F.
中科院分区：
文献类型：
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作者：
Guo, Yong;Lennon, Vanda A.;Lucchinetti, Claudia F.
重要性文件的肌肉病理兼容的靶向肌膜水通道蛋白-4（AQP 4）补体激活IgG意味着参与中枢神经系统以外的器官neuromyelocyte optica spectrum disorders.OBSERVATIONS我们报告一个51岁的妇女谁复发性视神经炎，横肌，AQP 4-IgG血清阳性，并经常性肌痛与高CK血症。肌肉活检显示散在肌纤维与内部核，萎缩和再生，但没有坏死。肌内膜和血管周围间隙中的轻度炎性渗出物由淋巴细胞、组织细胞和散在的嗜酸性粒细胞组成。肌膜表现出的损失AQP 4和沉积的IgG和补体激活产物，特征没有看到健康肌肉和免疫介导的myopathies.CONCLUSIONS和RELEVANCE控制活检样本伴随AQP 4-IgG血清阳性反映了骨骼肌AQP 4的致病性IgG的靶向高CK血症。自身免疫性AQP 4肌病的实体将视神经肌病谱扩展到中枢神经系统之外。
IMPORTANCE Documentation of muscle pathology compatible with targeting of sarcolemmal aquaporin-4 (AQP4) by complement-activating IgG implies involvement of organs beyond the central nervous system in neuromyelitis optica spectrum disorders.OBSERVATIONS We report on a 51-year-old woman who had relapsing optic neuritis, transverse myelitis, AQP4-IgG seropositivity, and recurrent myalgias with hyperCKemia. A muscle biopsy revealed scattered myofibers with internal nuclei, atrophy, and regeneration but no necrosis. Mild inflammatory exudates, in endomysial and perivascular spaces, consisted of lymphocytes, histiocytes, and scattered eosinophils. The sarcolemma exhibited loss of AQP4 and deposition of IgG and complement activation products, characteristics not seen in control biopsy samples of healthy muscle and immune-mediated myopathies.CONCLUSIONS AND RELEVANCE Recurrent hyperCKemia accompanying AQP4-IgG seropositivity reflects targeting of skeletal muscle AQP4 by pathogenic IgG. The entity of autoimmune AQP4 myopathy extends the neuromyelitis optica spectrum beyond the central nervous system.