Clinical Characteristics Of Neuromyelitis Optica Spectrum Disorders With Connective Tissue Diseases

Objective Neuromyelitis optica spectrum disorders(NMOSD)often coexist with connective tissue disorders(CTD).Compare clinical,laboratory and imaging features of NMOSD without and with CTD,and discuss different characteristics between two groups.Methods We reviewed the records of all NMOSD patients admitted to Tianjin Medical University General Hospital,Tianjin,China,from December 2014 to December 2017 and collected demographic information,clinical data,and blood specimens.NMOSD was diagnosed according to the 2015 international consensus diagnostic criteria for NMOSD,and CTD(e.g.,systemic lupus erythematosus(SLE),Sj?gren syndrome(SS),rheumatoid arthritis(RA)or undifferentiated CTD(UCTD))was diagnosed according to published rheumatology standards and guidelines.Inclusion criteria were as follows:(1)the serum samples of all the patients were tested for aquaporin 4 immunoglobulin G(AQP4-IgG),myelin oligodendrocyte glycoprotein immunoglobulin G(MOG-IgG),autoreactive antibodies(ANAs),extractable nuclear antigen autoantibodies(ENAs),rheumatoid factor(RF),and anti-neutrophil cytoplasmic antibodies(ANCAs),immunoglobulins(Ig),and complement(C);(2)spinal and brain magnetic resonance imaging(MRI)were available before high-dose intravenous methylprednisolone(IVMP)(1.0 g/d for 3 days)or intravenous immunoglobulin G(IVIG)(0.4 g/kg.d for 5 days);and(3)the time between study inclusion and the last relapse was more than 3 months.Tests for autoreactive antibodies(AQP4-IgG,MOG-IgG,ANAs,ENAs,RF and ANCAs),Ig,and C were conducted,along with other serology and cerebrospinal fluid index,in the immunology and clinical laboratory of our hospital.In the end,the total number of patients with NMOSD was 74,and 10 patients were excluded because the clinical data were incomplete.Further,the pathophysiology of MOG-IgG-related NMOSD was different from that of AQP4-IgG-positive NMOSD,7 cases who were MOG-IgG positive were excluded.57 patients with NMOSD were enrolled: 39 NMOSD patients without CTD and 18 NMOSD patients with CTD.Results 1.In terms of demographic and clinical characteristics,NMOSD patients with CTD had higher EDSS scores at nadir(P = 0.031)and more severe sensory dysfunction(P = 0.007)compared with NMOSD patients without CTD.2.In laboratory testing,compared with NMOSD patients without CTD,NMOSD patients with CTD had higher serum immunoglobulin G(IgG)(P = 0.013)and higher percentage of ?-globulin(P = 0.023);serum autoantibodies(such as ANA(P = 0.004),Anti-SSA(P < 0.001),Anti-Ro52(P < 0.001),Anti-SSB(P = 0.001)and RF(P = 0.012))were significantly elevated.3.In the MRI of the spinal cord,NMOSD with CTD was marked by longer spinal cord lesions(P = 0.018)and a lower frequency of short transverse myelitis(STM)than NMOSD without CTD(P = 0.010).NMOSD with CTD also featured more T1 hypointensity and T2 bright spotty lesions(T2 BSLs)on MRI than NMOSD without CTD(P = 0.001 and 0.011,respectively).4.In the MRI of the head,there was no significant difference in the lesions of the head between NMOSD without and with CTD.Conclusions A few characteristics differed between NMOSD with and without CTD.NMOSD patients with CTD had higher serum IgG,longer spinal cord lesions,a lower frequency of STM and more T1 hypointensity and T2 BSLs on spinal MRI than NMOSD patients without CTD.