视神经脊髓炎谱系疾病合并结缔组织病临床分析

目的 探讨视神经脊髓炎谱系疾病(NMOSD)合并结缔组织病(CTD)的临床特征。方法 回顾收集福建医科大学附属第二医院2015年1月至2020年2月住院患者NMOSD-CTD 16例及同期单纯NMOSD 54例,分别从首发表现、颅内病灶、脊髓病灶、实验室检查等方面对两组进行比较。结果 NMOSD-CTD组中合并干燥综合征(SS)的比例最高(10/16,62.5%)。与单纯NMOSD相比,NMOSD-CTD血清或脑脊液水通道蛋白4(AQP4)抗体阳性率明显增高(100%比70.2%,P=0.009),血清抗核抗体、抗干燥综合征A抗体、抗Ro52抗体等自身抗体阳性率也显著升高(P<0.001);扩展残疾状态量表评分≥6分者比例明显增高(50.0%比22.2%,P=0.035),两组疾病起病年龄、就诊年龄、复发次数、病程、颅内病灶分布、脊髓受累、激素冲击治疗有效率方面差异均无统计学意义(P均>0.05)。结论 NMOSD易合并CTD,其中以SS最常见,NMOSD-CTD的血清或脑脊液AQP4抗体阳性率、血清自身抗体阳性率高于单纯NMOSD,神经功能缺损程度更严重,应引起临床医生警惕。

Clinical Features of Neuromyelitis Optica Spectrum Disorders with Connective Tissue Diseases

Objective To investigate the clinical features of neuromyelitis optica spectrum disorders(NMOSD)with connective tissue diseases(CTD). Methods Clinical data of 16 NMOSD-CTD patients and 54 NMOSD patients admitted to the Second Affiliated Hospital of Fujian Medical University from January 2015 to February 2020 were collected.The initial symptom,intracranial lesion,spinal cord lesion,laboratory examination and treatment response were compared between the two groups. Results The incidence of Sjogren’s syndrome(SS)was the highest(10/16,62.5%)in NMOSD-CTD group.The NMOSD-CTD group had significantly higher positive rate of aquaporin-4 immunoglobulin G(AQP4-IgG)in serum or cerebrospinal fluid(100% vs. 70.2%,P=0.009),higher positive rates of serum anti-nuclear antibodies,anti Sjogren’s syndrome A antibodies and anti-Ro52 autoantibodies(P<0.001),as well as higher proportion of patients with the expanded disability status scale score ≥ 6(50.0% vs. 22.2%,P=0.035)than the NMOSD group.There was no significant difference between the two groups in the age of onset,visiting age,recurrence frequency,disease course,distribution of intracranial lesions,spinal cord involvement,or the effective rate of glucocorticoid pulse therapy(all P>0.05).Conclusions NMOSD is often complicated with CTD,and SS is the most common one.The positive rate of serum or cerebrospinal AQP4-IgG and the seropositivity of several other autoantibodies in NMOSD-CTD patients were higher than those in NMOSD patients.Neurological impairment in NMOSD-CTD patients were severer,which should arouse attention of clinicians.