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.

Objective To explore the clinical characteristics of postmenopausal patients with new-onset systemic lupus erythematosus(SLE).Methods The medical records of 289 patients with SLE in this hospital January 2015 to March 2021 were analyzed retrospectively.The patients were divided into the menopausal group(n=56)and the childbearing age group(n=205)according to whether they were menopausal at the time of onset.Male patients were divided into the male group(n=28).The differences in initial symptoms,clinical manifestations,laboratory indexes and SLE disease activity index(SLEDAI)scores among the three group were analyzed and compared.Results In the menopausal group,there were 5 cases aged 40-＜50 years old,31 cases aged 50-＜60 years old,14 cases aged 60-＜70 years old,and six cases aged ≥70 years old.37 cases occurred more than five years after menopause.Compared with the childbearing age group,the menopa-usal group had an older age of onset,a higher number of other diseases at the time of onset,a higher incidence of fever,edema,chest tightness and urgency as the first symptoms,weight loss,fatigue,myalgia and myasthe-nia,and a lower incidence of rash,kidney damage,WBC reduction,hemoglobin reduction,and of complement C3 and C4 reduction.The proportion of patients with SLEDAI score and SLEDAI score＞20 was lower(P＜0.05).Compared with the male group,the age of onset was older in the menopausal group,and the incidence of fever,edema,chest tightness and urgency as the first symptoms was higher,as well as the incidence of weight loss,myalgia and myasthenia,while the incidence of urinary surface foam as the first symptom and kid-ney damage were lower(P＜0.05).The most common laboratory abnormalities in postmenopausal group were ANA ≥1∶80,followed by anti-nucleosome antibody(+),anti-DS-DNA antibody(+),increased eryth-rocyte sedimentation rate,and decreased complement C3.Multivariate logistic regression analysis showed that compared with the menopause group,the risk of weight loss,myalgia and myasthenia was lower in the child-bearing age group,and the risk of kidney damage was higher(P＜0.05).Compared with the menopausal group,the male group had a higher risk of renal damage and a lower risk of complement C4 reduction(P＜0.05).Conclusion The first symptoms and clinical manifestations of postmenopausal SLE patients are atypi-cal,the organ damage is mild,and SLEDAI is low.

Objective:To analyze the differential expressions of ferroptosis related genes (FRGs) in proliferative lupus nephritis and its correlation with clinical indices (PLN) by analyzing the gene expression omnibus (GEO) database.Methods:The GSE65391 dataset was downloaded from GEO database, differential FRGs of PLN were screened by limma package of R language. Wilcoxon rank-sum test was used to analyze the differentially expressed genes in peripheral blood between 514 PLN patients and 72 healthy controls. Gene ontology (GO) and Kyoto encyclopedia of genes and genomes (KEGG) enrichment analysis of differential FRGs were determined with the clusterProfiler package. Hub genes were determined using random forest (RF), support vector machine (SVM), xgboost (XGB) and generalized linear model(GLM) machine learning algorithms. Finally, Spearman rank correlation analysis was used to analyze the correlation between hub genes and clinical indices.Results:A total of 38 differential FRGs of PLN patients were screened out, of which 16 were up-regulated and 22 were down-regulated. Biological process was enriched in cellular response to chemical stress, cellular component was enriched in transcription regulator complex, molecular function was enriched in DNA-binding transcription factor binding. KEGG enrichment analysis showed that FRGs were mainly involved in NOD-like receptor signaling pathway. GLM algorithm was selected to predict gene essentiality according to the area under the receiver operating characteristic (ROC) curve, 10 hub genes were determined, of which MYB was the most important. MYB was positively correlated with SLEDAI ( r=0.21, P<0.001), ALT ( r=0.20, P<0.001), AST ( r=0.18, P<0.001), LDH ( r=0.31, P<0.001), Cr ( r=0.24, P<0.001) and ESR ( r=0.22, P<0.001) and negatively correlated with albumin ( r=-0.28, P<0.001). Conclusion:FRGs may provide new insight into the potential mechanisms of the pathogenesis of PLN.

Polyarteritis nodosa (PAN) is a rare vasculitis that mainly involves small and medium arteries. It often occurs at the points where the vessels bifurcate, leading to microaneurysm formation, thrombosis, aneurysm rupture and bleeding, and infarction of organs.About a third of cases are associated with hepatitis B virus (HBV) infection.All tissues and organs of the body can be affected, with skin, joints and peripheral nerves being the most common.The pathological changes were fibrinoid necrosis, inflammatory cell infiltration and luminal thrombosis in the acute stage, and fibrous hyperplasia in the chronic stage.Overall outcomes for the disease have improved in recent decades, mainly reflecting early diagnosis and more effective treatments.The main treatments for PAN are glucocorticoid and cyclophosphamide.Patients with HBV-associated PAN should receive antiviral therapy and plasma exchange.

Objective To explore the status of glucocorticoid application in patients with systemic lupus erythematosus (SLE) in China.Methods Epidemiological survey was used.The SLE patients who met the 1997 classification criteria of American College of Rheumatology were enrolled.The usage of glucocorticoid and related adverse reactions were recorded and analyzed.Results A total of 400 SLE patients were enrolled,including 35 men and 365 women.The average age was (37.4 ± 14.0) years old,and the average duration of disease was (6.7 ± 5.8) years.There were 310 patients using glucocorticoid as maintenance.Sixty-one percent (n =244) patients started using medium dose (prednisone 30-＜ 60 mg/d) as the initial treatment of glucocorticoid,which lasted for (37 ± 11) days.The time of drug duration in patients with low dose prednisone (7.5-＜30 mg/d)and high dose (60-100 mg/d) was(92 ± 20)and (17 ± 3) days respectively (P ＜ 0.05 between 3 groups).However,patients receiving different initial dosage were of no discrepancy in the maintenance therapy.During maintenance,even though 51.0％ (n =158) patients were on prednisone 2.5-5 mg/d,the duration of drug use in ＞ 5-10 mg/d groupwas longer [(29.9 ± 3.3) months].Patients with involvement of internal organs had a higher tendency to use 60-100 mg/d prednisone or pulse-dose therapy in the initial treatment,nevertheless these two groups had no difference of maintenance dosage.Among all 400 patients,62 patients withdrew glucocorticoid,including 17 patients with disease remission (4.3％),44 by self-withdrawal and one with adverse reaction.Conclusion In China,the medium dosage of glucocorticoid is the most common initial treatment in patients with SLE.Prednisone 2.5-5 mg/d was the most common choicefor maintenance therapy.Currently,the proportion of glucocorticoid withdrawal remains low in SLE patients achieving remission.

Objective To investigate the current situation in Chinese rheumatologic physicians' clinical diagnosis and evaluation of Takayasu's arteritis (TA).Methods Nineteen rheumatology experts and three vascular surgery specialists in China were invited to make the nationwide investigation for the first time about the diagnosis and disease activity evaluation of TA in China,through the questionnaire survey on the internet.Weighted average was used to calculate the average scores of corresponding problems.Results Chinese experts mainly adopted 1990 American College of Rheumatology (ACR) classification criteria for clinical diagnosis of TA.In details,symptoms of age,limb claudication and amaurosis,signs including pulselessness or pulse weakening,vascular bruits,increasing bilateral pulse pressure and hypertension and acute phase reactants (APR) were critical to the clinical diagnosis of TA.Besides,noninvasive imaging examinations,such as computed tomography angiography (CTA),magnetic resonance angiography (MRA),vascular ultrasonography,and positron emission tomography (PET) were also of great importance.In the aspect of disease activity assessment,Chinese experts mainly used Kerr scoring tool.APR and noninvasive radiological examinations were considered with vital value.Some TA patients with carotid artery involvement were recommended using vascular ultrasonography,while others with pulmonary artery and thoracic/abdominal aorta trunk involvement were preferred CTA other than MRA.Conclusions APR and noninvasive imaging examinations were thought with great help to make clinical diagnosis and evaluation of TA for Chinese physicians.

Objective To study the MR performances of finger joints with health subjects and rheumatoid arthritis (RA)patients,and to detect the imaging quality of a custom-made finger coil.Methods Finger coil,extremity coil,flexible surface coil and 3 inch surface coil were used to scan the self-made water phantom with similar T2 WI sequence,then the signal to noise ratio (SNR)on single image was measured for the comparison.The middle finger specimen (1 finger)was used for the research,5 healthy volunteers (8 fingers)and 18 RA patients (22 fingers)were also scanned.Results The SNR of finger coil was over 1.5 times higher than that of the 3 inch surface coil and 4 times higher than that of the extremity coil and flexible surface coil.For the 22 fingers with confirmed RA,synovitis was found in 1 7,joint effusion in 10,bone marrow edema in 5,bone erosion in 7 and tenosynovitis in 6.Conclusion The finger coil has higher SNR.

BACKGROUNDLupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus. Although there have been substantial improvements in LN treatment over the last decade, the outcome remains unoptimistic in a considerable percentage of patients. The aim of this study was to evaluate the efficacy and safety of mizoribine (MZR), a novel selective inhibitor of inosine monophosphate dehydrogenase, as induction treatment for active LN in comparison with mycophenolate mofetil (MMF) and intravenous cyclophosphamide (CYC).

METHODSNinety patients with active LN were observed. Thirty patients were given MZR orally at the dose of 300 mg every other day. Thirty patients took MMF at 2 g per day in two divided doses. Thirty patients received CYC intravenously 0.5 g every 2 weeks. Therapeutic effects and adverse events (AEs) were evaluated at the end of 24-week treatment. One-way analysis of variance (ANOVA) followed by Dunn's test was applied to compare the difference among the groups. For comparing categorical data between two groups, χ(2) test was employed.

RESULTSEarly responses at week 12 were achieved by 73.3%, 90.0%, and 96.7% in MZR, MMF, and CYC groups, respectively. There was no significant difference in the complete remission rates (22.7%, 24.0%, and 25.0%, respectively) or overall response rates (68.2%, 72.0%, and 75.0%, respectively) among the three groups at week 24. The most prominent drop-down of Systemic Lupus Erythematosus Disease Activity Index scores was observed in MMF or CYC group, and the decline of health assessment questionnaire scores in MZR or MMF group was more prominent than that in the CYC group at week 12. Serum complement 3 (C3) or C4 levels were elevated in all groups after the treatments. CYC was more effective in inhibiting anti-double-stranded DNA antibody, while MZR was more effective in inhibiting antinuclear antibody. The incidences of AEs in patients treated with CYC were significantly higher than those in patients treated with MZR or MMF (24.2% for CYC vs. 3.3% for MZR, and 2.6% for MMF, P = 0.01).

CONCLUSIONSMZR is well tolerated and has an effect similar to MMF in the induction therapy of active LN. MZR may serve as an alternative approach for LN patients.

Administration, Intravenous ; Adolescent ; Adult ; Aged ; Cyclophosphamide ; administration & dosage ; therapeutic use ; Enzyme Inhibitors ; administration & dosage ; therapeutic use ; Female ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lupus Nephritis ; drug therapy ; Male ; Middle Aged ; Mycophenolic Acid ; administration & dosage ; therapeutic use ; Ribonucleosides ; administration & dosage ; therapeutic use ; Treatment Outcome ; Young Adult

Objective To investigate the efficacy and adverse reactions of half-dose glucocorticosteroid and cytoxan,combined with leflunomide for the treatment of lupus nephritis (LN) of MRL/lpr mice,and provide experimental evidences for LN therapy.Methods Twenty-eight 10-week-old MRL/lpr mice were randomly divided into four groups:Group A,blank control group; Group B,classical control group; Group C,full-dose control group; Group D,half-dose treatment group,with 7 mice in each group.The therapeutic efficacy and side reactions in the four groups were observed and compared before and 12 weeks after treatment.Statistical analysis was conducted with one-way ANOVA,q test and Pearson's correlation analysis.Results The serum anti-double stranded DNA (anti-dsDNA) antibody titers (0.43±0.16,0.32±0.09,0.44± 0.18,1.95±0.19) U/ml,serum creatinine level (1.63±0.63,0.40±0.23,0.82±0.21,10.86±2.17) mg,24-hour urine protein excretion level (71±8,60±5,68±3,121±10) μmol/L and renal pathological changes in group B,C,D were significantly improved than those of the group A (P＜0.05) after 12 weeks treatment.There was no significant difference in the efficacy between group B,C,and D (P＞0.05).The incidence of adverse reactions in group D was significantly lower than that in other groups (P＜0.05).Conclusion Multi-target therapy,such as half-dose prednisone and CTX,combined with leflunomide can effectively control lupus disease activity with less side effects.This regimen is cheap,safe and effective for the treatment of LN in MRUL/lpr mice.This study has provided animal evidences for this multi-target therapy for LN.

ObjectiveTo investigate the association of complement C3 with clinical and serological features of patients with systemic lupus erythematosus.MethodsData was collected by the same methods in the past ten years in fifteen hospitals in Jiangsu Province and then data weres summarized for retrospective analysis.Clinical and laboratory data were selected and then analyzed by Chi-square test,Wilcoxon rank sum test and Logistic regression.ResultsOne thousand four hundred and five patients were investigated.One thousand and forty two had low serum complement C3 level.In this case control study,hospitalization age,disease course,admission times,pleurisy,gastrointestinal involvement,general lymphadenopathy/hepatosplenomegaly,white blood cell count, haemoglobin level,platelet count, serum C-reactive protein level,serum albumin level,serum creatinine level,Urine protein quantification,anti-nuclear antibodies (ANA),anti-dsDNAantibodies, anti-SmantibodiesandSLEDAIscore were possible factors associatedwith complement C3 reduction(P＜0.05).Logistic regression analysis showed that CRP (OR=0.396,0.254-0.617,P=0.000),ANA (OR=2.907,1.267-6.670,P=0.012),urine protein level(OR=1.702,1.043-2.779,P=0.033) and SLEDAI score (OR-0.930, 0.886-0.975,P-0.003) were correlated with complement C3 reduction.Conclusion Complement C3 level is valuable for lupus flare assessment.The complement C3 reduction is a risk factor for renal impairment.