Different Clinical Characteristics Of Longitudinal Extensive Transverse Myelitis With And Without Connective Tissue Diseases

Objective Longitudinally extensive transverse myelitis(LETM)often combined with connective tissue disorders(CTD),especially Sj(?)gren syndrome(SS)and systemic lupus erythematosus(SLE).The purpose of this study is to explore the similarities and differences between LETM patients with and without CTD in demographic information,clinical manifestations,laboratory tests,imaging features.Methods We enrolled 92 LETM patients who admitted to Tianjin Medical University General Hospital,Tianjin,China,from October 2014 to October 2019 and collected demographic information,clinical manifestations,laboratory examination results and imaging data in detail.All the data were analyzed by SPSS 22.0 software.All quantized data were expressed as mean ± standard deviation or median and range.T test or Mann Whitney U test was used for measurement data,chi square test or Fisher accurate test was used for counting data.P value < 0.05 was statistically significant.Results1.The majority of LETM patients were women(83/92,90.2%),most of them had a tendency to relapse(70/92,76.1%),SS was the most common type of CTD.2.LETM patients with CTD had higher EDSS scores at nadir and more severe sensatory dysfunction than LETM patients without CTD(P = 0.002,P = 0.002),we got similar results in the LETM patients who were AQP4-Ab positive with CTD(P =0.026,P = 0.006).3.The positive rate of autoimmune antibodies such as anti-SSA / Ro52 / SSB,RF in LETM with CTD was higher,and serum Ig G,Ig A,γ globulin levels were elevated than those in LETM without CTD.The above differences are more obvious in AQP4-Ab positive LETM with CTD.4.The thoracic spinal cord was the most common spinal cord lesion(77/92,83.7%).However,there was no statistical difference between the LETM patients with or without CTD in the affected parts of the brain and spinal cord.The average number of spinal cord lesions of LETM were not statistically different between the two groups.5.Age at CTD of SS group was older than the group of patients with other CTD.LETM patients with SS tended to LETM first with subsequent CTD.The levels of IgG,IgA and γ globulin were higher than the other CTD group,but ALB was lower.Conclusions There were different clinical characteristics of LETM patients with and without CTD.LETM patients with CTD had higher EDSS scores at nadir,heavier sensory dysfunction.What’s more,the proportion of abnormalities in autoimmune antibodies was higher,and the levels of Ig G,Ig A,and γ globulin were elevated in LETM with CTD group.We also find similar results in AQP4-Ab positive LETM patients with CTD.LETM patients with SS had higher levels of Ig G,Ig A,and γ globulin than other CTD group,however,ALB was lower.