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埃及 NMOSD 患者的水通道蛋白 4-IgG 状态及其对临床特征和治疗反应的影响。

The aquaporin4-IgG status and how it affects the clinical features and treatment response in NMOSD patients in Egypt.

机构信息

Department of Neurology, Kasr-Alainy Faculty of Medicine, Cairo University, 7 Emtedad al Ikhaa, Maadi, Nile Corniche, Cairo, Egypt.

Department of Clinical Pathology, Kasr-Alainy Faculty of Medicine, Cairo University, Cairo, Egypt.

出版信息

BMC Neurol. 2021 Feb 3;21(1):53. doi: 10.1186/s12883-021-02083-1.

Abstract

BACKGROUND

In Egypt, the characterization of Neuromyelitis Optica Spectrum Disorder (NMOSD) is lacking.

OBJECTIVES

To determine the demographics, clinical features, aquaporin4 antibodies (AQP4-IgG) status, and neuroimaging of Egyptian NMOSD patients.

METHODS

Retrospective analysis of 70 NMOSD patients' records from the MS clinic, Kasr Alainy hospital, between January 2013 and June 2018.

RESULTS

Patients' mean age was 34.9 ± 9.2 years, and the mean at disease onset was 28.9 ± 10.5 years. Fifty-nine patients had an initial monosymptomatic presentation. AQP4-IgG was measured using either enzyme-linked immunosorbent assay (ELISA) (22 patients) or cell-based assay (CBA) (34 patients). Six and 29 patients had positive results, respectively (p < 0.001). 84% had typical NMOSD brain lesions. Longitudinally extensive myelitis was detected in 49 patients, and 9 had either short segments or normal cords. Treatment failure was higher in seropositive patients. Rituximab significantly reduced the annualized relapse rate (ARR) compared to Azathioprine with a percentage reduction of (76.47 ± 13.28) and (10.21 ± 96.07), respectively (p = 0.04). Age at disease onset was the only independent predictor for disability (p < 0.01).

CONCLUSION

Treatment failure was higher in seropositive patients. However, there was no difference in clinical or radiological parameters between seropositive and seronegative patients. Patients, who are polysymptomatic or with older age of onset, are predicted to have higher future disability regardless of the AQP4-IgG status.

摘要

背景

在埃及,视神经脊髓炎谱系疾病(NMOSD)的特征尚不清楚。

目的

确定埃及 NMOSD 患者的人口统计学、临床特征、水通道蛋白 4 抗体(AQP4-IgG)状态和神经影像学特征。

方法

对 2013 年 1 月至 2018 年 6 月在 Kasr Alainy 医院多发性硬化症(MS)诊所就诊的 70 例 NMOSD 患者的病历进行回顾性分析。

结果

患者的平均年龄为 34.9±9.2 岁,发病时的平均年龄为 28.9±10.5 岁。59 例患者有首发单症状表现。AQP4-IgG 采用酶联免疫吸附试验(ELISA)(22 例)或细胞基础检测法(CBA)(34 例)进行检测。分别有 6 例和 29 例患者检测结果为阳性(p<0.001)。84%的患者有典型的 NMOSD 脑病变。49 例患者有长节段脊髓炎,9 例患者有短节段或正常脊髓。血清阳性患者的治疗失败率更高。与硫唑嘌呤相比,利妥昔单抗可显著降低年复发率(ARR),降低幅度分别为(76.47±13.28)%和(10.21±96.07)%(p=0.04)。发病年龄是残疾的唯一独立预测因素(p<0.01)。

结论

血清阳性患者的治疗失败率更高。然而,血清阳性和阴性患者的临床或影像学参数无差异。无论 AQP4-IgG 状态如何,首发症状多或发病年龄较大的患者,未来残疾的风险更高。

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