摘要
目的总结原发性干燥综合征(PSS)合并视神经脊髓炎谱系疾病(NMOSD)的临床特点,提高对PSS合并NMOSD的认识。方法回顾性分析2013年6月至2019年8月于郑州大学第一附属医院住院诊治的22例PSS合并NMOSD患者的临床资料、实验室检查、影像学表现、治疗及随访1 a复发情况等。结果(1)22例PSS合并NMOSD患者中,女19例,男3例,年龄21~64(44.5±11.57)岁;中位病程27.5个月(10 d^96个月)。(2)20例患者以中枢神经系统受累为首发表现,其中视神经炎5例,脊髓炎12例,脑部病变3例。(3)脊髓磁共振成像:颈髓受累6例,胸髓受累5例,胸髓及腰髓同时受累8例,无腰髓受累者。(4)头颅磁共振成像:脑部病变10例,延髓病变2例,脑及延髓病变4例。(5)行血清水通道蛋白4(AQP4)抗体检测20例,阳性17例(85%);行脑脊液AQP4抗体检测9例,阳性7例(77.8%)。血清AQP4抗体阴性患者脑部病变发生率为100%(3/3),血清AQP4抗体阳性患者脑部病变发生率为76.5%(13/17)。(6)22例患者均接受自身抗体检测,其中抗核抗体(ANA)阳性率为72.7%,抗SSA抗体阳性率为100%,抗SSB抗体阳性率为18.2%。结论PSS合并NMOSD患者以女性为主,脊髓病变多见且以长节段受累为主,AQP4抗体及抗SSA抗体阳性率高。AQP4抗体阴性患者脑部受累发生率高,头颅磁共振成像可能有助于诊断。
Objective To summarize the clinical characteristics of primary Sjogren syndrome(PSS)combined with neuromyelitis optica spectrum diseases(NMOSD),and to improve the understanding of PSS with NMOSD.Methods A retrospective analysis of the clinical data,laboratory examinations,imaging findings and treatment of 22 patients with PSS combined with NMOSD who were hospitalized in the First Affiliated Hospital of Zhengzhou University from June 2013 to August 2019.Results(1)There were 22 patients with PSS and NMOSD,19 females and 3 males,aged 21-64(44.5±11.57)years;median duration of disease was 27.5 months(10 days-96 months).(2)Twenty patients had central nervous system as the first manifestation,including 5 cases of optic neuritis,12 cases of myelitis,and 3 cases of brain lesions.(3)Magnetic resonance imaging of the spinal cord:6 cases of cervical spinal cord involvement,5 cases of thoracic spinal cord involvement,8 cases both thoracic and lumbar spinal cord involvement,and no lumbar spinal cord involvement.(4)Magnetic resonance imaging of brain:showed brain lesions in 10 cases,medulla oblongata in 2 cases,brain and medulla oblongata in 4 cases.(5)20 cases underwent serum aquaporin 4(AQP4)antibody test,17 cases(85%)were positive;9 cases underwent CSF AQP4 antibody test,7 cases(77.8%)were positive.The incidence of brain lesions in patients with serum AQP4 antibody negative was 100%(3/3),which was 76.5%(13/17)with serum AQP4 antibody positive.(6)All patients were tested for auto-antibodies.The positive rates of antinuclear antibody(ANA),anti-SSA antibody,anti-SSB antibody were 72.7%,100%and 18.2%,respectively.Conclusion The patients with PSS and NMOSD are mainly females.Spinal cord lesions are more common and are mainly affected by long segments.The positive rates of AQP4 antibody and anti-SSA antibody are high.AQP4 antibody-negative patients have a high incidence of brain involvement,and brain magnetic resonance imaging maybe helpful for diagnosis.
作者
朱政言
汪晴
张钰琦
孙金磊
刘升云
ZHU Zheng-yan;WANG Qing;ZHANG Yu-qi;SUN Jin-lei;LIU Sheng-yun(Department of Rheumatology and Immunology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,China)
出处
《河南医学研究》
CAS
2020年第36期6744-6747,共4页
Henan Medical Research
