AQP4-IgG autoimmunity in Japan and Germany: Differences in clinical profiles and prognosis in seropositive neuromyelitis optica spectrum disorders.
AQP4-IgG autoimmunity in Japan and Germany: Differences in clinical profiles and prognosis in seropositive neuromyelitis optica spectrum disorders.
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DOI：
10.1177/20552173211006862
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发表时间：
2021-04
影响因子：
--
通讯作者：
Zimmermann HG
中科院分区：
文献类型：
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作者：
Asseyer S;Masuda H;Mori M;Bellmann-Strobl J;Ruprecht K;Siebert N;Cooper G;Chien C;Duchow A;Schließeit J;Liu J;Sugimoto K;Uzawa A;Ohtani R;Paul F;Brandt AU;Kuwabara S;Zimmermann HG
视神经肌病谱系障碍（NMOSD）的临床结局在不同地区各不相同。描述日本和德国NMOSD患者的临床特征。回顾性分析日本（n = 54）和德国（n = 38）的水通道蛋白4-免疫球蛋白G（AQP 4-IgG）阳性NMOSD患者的病历。两个队列的残疾状态相似，尽管日本患者的病程较长（13.3 ± 11.1 vs 8.1 ± 6.9年，p = 0.018），但复发率相似。视神经炎和肌萎缩是两个队列中最常见的发作。日本患者的脑发作发生率更高（40.7% vs. 15.8%，p = 0.020）。在日本队列中，从疾病发作到首次免疫治疗开始的时间（中位数[四分位距] 2.3 [0.3-10.1] vs. 0.6 [0.2-1.9]年，p = 0.009）和发作次数（2.5 [1-7] vs. 2 [1-3]，p = 0.047）更高。利妥昔单抗是德国队列中最常见的药物（52.6%），而在日本队列中未使用（p < 0.001），其中口服泼尼松龙是最常见的药物（92.6% vs. 15.8%，p < 0.001）。德国队列中自身免疫性合并症的频率较高（39.5% vs. 18.5%，p = 0.047）。与日本NMOSD患者相比，德国患者的残疾相似，尽管病程较短，免疫抑制剂治疗更早和更频繁。
Clinical outcomes in neuromyelitis optica spectrum disorders (NMOSD) vary across different regions. To describe clinical profiles in Japanese and German NMOSD patients. Medical records of aquaporin-4-immunoglobulin G (AQP4-IgG) positive NMOSD patients from Japan (n = 54) and Germany (n = 38) were retrospectively analyzed. The disability status was similar between both cohorts, although Japanese patients had a longer disease duration (13.3 ± 11.1 vs. 8.1 ± 6.9 years, p = 0.018) but similar relapse rates. Optic neuritis and myelitis were the most frequent attacks in both cohorts. Brain attacks occurred more frequently in Japanese patients (40.7% vs. 15.8%, p = 0.020). The time from disease onset (median [interquartile range] 2.3 [0.3-10.1] vs. 0.6 [0.2-1.9] years, p = 0.009) and the number of attacks (2.5 [1-7] vs. 2 [1-3], p = 0.047) until start of the first immunotherapy were higher in the Japanese cohort. Rituximab was the most common drug in the German cohort (52.6%) and not given in the Japanese cohort (p < 0.001), where oral prednisolone was the most common drug (92.6% vs. 15.8%, p < 0.001). The frequency of autoimmune comorbidities was higher in the German cohort (39.5% vs. 18.5%, p = 0.047). Compared with Japanese NMOSD patients, German patients presented with similar disability despite shorter disease duration and earlier and more frequent immunosuppressive therapy.