Clinical characteristics of patients with neuromyelitis optica spectrum disorders with other autoantibodies

Abstract

Objective To analyze the clinical characteristics of patients with neuromyelitis optica spectrum disorders (NMOSDs) with or without autoantibodies other than aquaporin 4 (AQP4)⁃IgG. Methods A total of 90 NMOSDs patients were included from September 2014 to June 2017. First symptoms, first diagnosis, monophasic or polyphasic course of disease, comorbidities, Expanded Disability Status Scale (EDSS) score, treatment regimen and annualized relapse rate (ARR) were recorded. Laboratory tests for cerebrospinal fluid (CSF) white blood cell count and protein quantification, as well as serum autoimmune⁃related antibodies, including anti⁃nuclear antibody (ANA), A type and B type Sjögren's syndrome antibody (SSAandSSB), thyroid peroxidase (TPO) and thyroglobulin (TG) antibodies, anti⁃double stranded DNA antibody (dsDNA), perinuclear antineutrophil cytoplasmic antibody (pANCA), anti⁃endothelial cell antibody (AECA), Ro⁃52 antibody, Jo⁃1 antibody, anti⁃cardiolipin antibody (ACA), anti⁃mitochondria antibody M2 subtype (AMA⁃M2), human leukocyte antigen⁃B27 (HLA⁃B27), and myelin oligodendrocyte glycoprotein (MOG) antibody were examined. Brain and whole spine MRI were performed to observe the lesion site, distribution and length of spinal cord lesions. Results Among the 90 patients, 34 (37.78%) were accompanied by autoimmune⁃related antibodies(positive group),including 19 (55.88%) ANA positive cases, 13 (38.24%) SSA positive cases, 12 (35.29%) TPO antibody positive cases, and 10 (29.41%) TG antibody positive cases. The remaining 56 patients (62.22%) were not accompanied by autoimmune⁃related antibodies (negative group). In both groups, optic neuritis [52.94% (18/34) vs. 58.93% (33/56)], brain symptoms [26.47% (9/34) vs. 17.86% (10/56)] and myelitis [14.71% (5/34) vs. 21.43% (12/56)] were the main initial symptoms (Fisher exact probability: P =0.504). There were no significant differences in the firstdiagnosis(χ2=1.634, P=0.201),monophasicorpolyphasiccourse(Fisherexactprobability: P=1.000), comorbidities(χ2=1.275, P=0.302),EDSS score at the peak of the first onset (Z=⁃0.747, P=0.455) and at the inclusion (Z=⁃0.379, P=0.705), treatment plan (χ2=0.662, P=0.416) and ARR (Z=⁃0.370, P= 0.711). There was no statistically significant difference in CSF white blood cell count (Z =⁃1.163, P = 0.245) and protein quantification (Z =⁃0.340, P =0.734). MRI showed that 68 cases (75.56%) had intracranial lesions [28 cases (82.35%) in the positive group and 40 cases (71.43%) in the negative group], mainly located in the parietal lobe, frontal lobe and lateral ventricle. There were 62 cases (68.89%) with spinal lesions [21 cases (61.76%) in the positive group and 41 cases (73.21%) in the negative group], which were mainly cervical and thoracic myeloid lesions and medium⁃length segment lesion. There were no statistically significant differences in intracranial lesions (χ2=1.367, P=0.242), spinal cord lesions (χ2= 1.294, P=0.255) or lesion length (Z=⁃0.647, P=0.517) between 2 groups. Conclusions Therewereno significant differences between NMOSDs patients with and without other autoimmune⁃related antibodies in terms of initial symptoms, CSF white blood cell count and protein quantification, MRI lesion distribution characteristics, etc. The value of autoimmune⁃related antibodies in clinical characteristics of NMOSDs patients remains to be further explored. DOI：10.3969/j.issn.1672⁃6731.2020.09.007