ABSTRACT
Objective:To investigate the clinical features and prognosis of male with anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody.Methods:The clinical data of 246 patients with DM and anti-MDA5 autoantibodies hospitalized by Jiangsu Myositis Cooperation Group from 2017 to 2020 were collected and retrospectively analyzed. Chi-square test was performed to compared between counting data groups; Quantitative data were expressed by M ( Q1, Q3), and rank sum test was used for comparison between groups; Single factor survival analysis was performed by Kaplan-Meier method and Log rank test; Cox regression analysis were used for multivariate survival analysis. Results:①The male group had a higher proportion of rash at the sun exposure area [67.1%(47/70) vs 52.8%(93/176), χ2=4.18, P=0.041] and V-sign [50.0%(35/70) vs 30.7%(54/176), χ2=8.09, P=0.004] than the female group. The male group had higher levels of creatine kinase [112(18, 981)U/L vs 57 (13.6, 1 433)U/L, Z=-3.50, P<0.001] and ferritin [1 500 (166, 32 716)ng/ml vs 569 (18, 14 839)ng/ml, Z=-5.85, P<0.001] than the female group. The proportion of ILD [40.0%(28/70) vs 59.7%(105/176), χ2=7.82, P=0.020] patients and the red blood cell sedimentation rate[31.0(4.0, 101.5)mm/1 h vs 43.4(5.0, 126.5)mm/1 h, Z=-2.22, P=0.026] in the male group was lower than that of the female group, but the proportion of rapidly progressive interstitial lung disease (PR-ILD) [47.1%(33/70) vs 31.3%(55/176), χ2=5.51, P=0.019] was higher than that of the female group. ②In male patients with positive anti-MDA5 antibodies,the death group had a shorter course of disease[1.0(1.0, 3.0) month vs 2.5(0.5,84) month, Z=-3.07, P=0.002], the incidence of arthritis [16.7%(4/24) vs 42.2%(19/45), χ2=4.60, P=0.032] were low than those in survival group,while aspartate aminotransferase (AST)[64(22.1, 565)U/L vs 51(14,601)U/L, Z=-2.42, P=0.016], lactate dehydrogenase (LDH) [485(24,1 464)U/L vs 352(170, 1 213)U/L, Z=-3.38, P=0.001], C-reactive protein (CRP) [11.6(2.9, 61.7) mg/L vs 4.95(0.6, 86.4) mg/L, Z=-1.96, P=0.050], and ferritin levels [2 000(681, 7 676) vs 1 125 (166, 32 716)ng/ml, Z=-3.18, P=0.001] were higher than those in the survival group, and RP-ILD [95.8%(23/24) vs 22.2%(10/45), χ2=33.99, P<0.001] occurred at a significantly higher rate. ③Cox regression analysis indicated that the course of disease LDH level, and RP-ILD were related factors for the prognosis of male anti-MDA5 antibodies [ HR (95% CI)=0.203(0.077, 0.534), P=0.001; HR (95% CI)=1.002(1.001, 1.004), P=0.003; HR (95% CI)=95.674 (10.872, 841.904), P<0.001]. Conclusion:The clinical manifestations of male anti-MDA5 antibody-positive patients are different from those of female. The incidence of ILD is low, but the proportion of PR-ILD is high. The course of disease, serum LDH level, and RP-ILD are prognostic factors of male anti-MDA5 antibody-positive patients.
ABSTRACT
ObjectiveTo investigate the number of γδT cells in the peripheral blood from patients with systemic lupus erythematosus(SLE) and their correlation to disease activity.MethodsγδT cells were detected in the peripheral blood from 42 SLE patients and 20 normal controls by flow cytometry.Anniex-V/Pl double-staining flow cytometry was employed to observe the proportion of the apoptotic γδ and CD3+ T cells in 6 SLE patients with active disease and 6 normal controls,respectively.The levels of plasma anti-nuclear antibody and anti-dsDNA antibody were determined by using enzyme-linked immunosorbent assay.Data were analyzed with t test and Pearson's correlation test.ResultsThe percentages of γδT cells were remarkably down-regulated in SLE patients [(3.0±1.8)％ ] with active disease compared with that of those patients with inactive[(5.3±3.0)％] disease and normal controls [(6.8±2.8)％](t=-3.071 and -5.913 respectively,both P＜0.01 ).The absolute number of γδT cells decreased significantly in SLE patients with active disease[ ( 1.7± 1.6)× 107/L ] than those with inactive SLE [ (5.3±3.6)× 107/L ] (t=-3.292,P＜0.01 ),and both were lower than the normal controls [ (10.1±5.0)×l 07/L] (t=-7.247 and -2.905 respectively,both P＜0.01 ).There was a negative correlation between systemic lupus erythematosus disease activity index (SLEDAI) andT cell counts in 30 SLE patients with active disease (r=-0.365,P=0.047).γδT cell percentage (r=-0.336,P=0.030) and counts (r=-0.410,P=0.007) were both inversely correlated with the erythrocyte sedimentation rate,but positive correlation were found between hemoglobulln and γδT cell counts (r=0.409,P=0.007).The apoptosis ofγδT cells in SLE patients was more common than in normal controls(t=2.886,P＜0.05 ).The number of apoptotic γδT cells was higher than that of CD3+ T cells in SLE patients (t=2.952,P＜0.05 ).Conclusion γδT cells of the peripheral blood of SLE patients are down-regulated partially due to excessive apoptosis,which may correlate with the disease activity.
ABSTRACT
Objective To investigate the expression levels of interferon-inducible genes (IFIT1,IFIT4,OAS1,OASL,ISG15) in the peripheral blood mononuclear cells (PBMCs) of patients with systemic lupus erythematosus(SLE).and the relations between these genes expression levels and disease activity are explored.Methods Sybr green dye based real-time quantitative PCR method was used to detect the expression levels (indicated as-△△Ct value) of WIT1,IFIT4.OAS1,OASL and ISG15 in 76 patients with SJJE and 54 controls.Their expression levels were compared with erythroeyte sedimentation rate (ESR),serum C reactive protein (CRP),complement C3,C4.antinuclear antibody (ANA).anti-double stranded DNA antibody.The associations between the expression levels of IFIT1,IFIT4,OASI.OASL,ISG15,ESR,CRP,complement C3,C4,ANA,anti-double stranded DNA antibody and SLEDAI scores in patients with SLE were analyzed.Results ① The expression levels of WIT1,IFIT4,OAS1,OASL and ISG15 in the SLE patients were significantly higher than those of the normal controls (P＜0.01).The expression levels of IFIT1,IFIT4,OAS1,OASL and ISG15 in active SLE patients were higher than those of inactive SLE patients (P＜0.05).The real time expression levels of IFIT1,IFIT4,OAS1.OASL and ISG15 showed positive correlations with each other (r＞0.5,P＜0.05) in patients with SLE.② The expression levels of IFIT1,IFIT4,OAS1,OASL and ISG15 were positively correlated with the SLEDAI scores (r＞0.5,P＜0.05).③ There was no correlation between ESR,CRP,complement C3,C4,ANA and the expression levels of IFIT1,IFIT4,OAS1,OASL,ISG15,SLEDAI scores except anti-double stranded DNA antibody (r＞0.5.P＜0.05).Conclusion The expression levels of IFIT1,IFIT4,OAS1,OASL and ISG15 in patients with SLE are significantly higher than those of the normal controls,and positively associated with SLEDAI scores,so they are helpful in evaluating SLE disease activity and severity.IFIT1,IFIT4,OAS1,OASL and ISG15 genes may be the potential treating targets for SLE.