Objective In 2016,European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric rheumatology international trials organization (PRINTO) released the classification criteria for macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA).Due to the similarities of both clinical manifestations and pathogenesis between adult-onset Still dsease (AOSD) and sJIA,we hope to evaluate the 2016 sJIA-AMS classification in AOSD patients.Methods A total of 169 AOSD patients who were hospitalized in Renji Hospital were enrolled in this study.AOSD patients were divided into AOSD with MAS and AOSD without MAS,using the 2016 sJIA-MAS criteria.The data of the two groups were analyzed by Chi-square test,Mann-Whitney U test and binary Logistic analysis,and factors influencing the prognosis of patients were analyzed by Kaplan-Meier and COX regression analysis.Results According to sJIA-MAS criteria,56 AOSD patients with MAS were identified in all the 169 AOSD cases.In AOSD patients,the incidence of splenomegaly and pericarditis/myocarditis was significantly higher in patients with MAS than in AOSD without MAS [42.9％ vs 14.2％,OR(95％CI)=4.50(2.13,9.51),P＜0.01;10.7％ vs 0.9％,OR(95％CI)=13.21 (1.56,113.57),P＜0.01],also the incidence of liver dysfunction was higher in AOSD with MAS [67.8％ vs 11.5％,OR(95％CI)=0.18(7.26,36.33),P＜0.01].Among the AOSD with MAS,62.5％(35/56) of these patients received large-dose glucocorticoid therapy,5.4％ (3/56) received the glucocorticoid pulse therapy,48.2％(27/56) were treated with IVIG,and 26.8％(15/56) were treated with calcium phosphatase inhibitors.The mortality rates of AOSD with MAS was 8.9％(5/56),which was significantly higher than 1.8％(2/113) (OR =5.44,P＜0.05),the mortality rate of the AOSD without MAS.Patients who fulfilled the sJIA-MAS criteria suggested poor prognosis (OR=0.041,P=5.44),and the platelet count ≤ 181× 109/L (OR=12.17,P=0.002),alanine aminotransferase ＞48 U/L (OR=9.43,P=9.040) were also highly suggestive of poor prognosis.Conclusion The 2016 sJIA-MAS classification criteria are particularly valuable for early recognization of MAS in AOSD patients,and convenient to use.AOSD patients fulfilled sJIA-MAS criteria are more severe,and require larger doses of glucocorticoid and more immunosuppression therapy compared to patients without MAS,and the prognosis of these patients is also poor.

Objective To investigate the clinical characteristics and current condition of treatment for systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH).Methods 10-year inpatients cases were reviewed and followed up.Cases were divided into 2 groups:group A:patients with baseline pulmonary arterial systolic pressure (PASP) lower than 70 mmHg； group B:patients with baseline PASP higher than 70 mm Hg.Pearson's correlation analysis,Chi-square test,Logistic regression,Cox-Mantel and Wilcoxon test were used for statistical analysis.Results There were totally 155 cases with 184 records of admission which accounted for 4.16％ among total lupus cases.The main clinical characteristics included Raynaud's phenomenon (47.3％),pericardial effusion (41.9％) and high titer of anti-RNP antibody (55.4％).There were 132 cases enrolled for prognostic statistical analysis.There were 47 cases of death (35.6％) in total,among which 9 cases (19.1％) were in group A and 38 cases (80.9％) were in group B.In group A,there was a positive correlation between PASP and lupus disease activity index score.Single therapy analysis by Chi-square test showed that cyclophosphamide (CTX) (P＜0.05) and PAH targeted drugs (P＜0.01) were significantly associated with favorable outcome but logistic regressive analysis only confirmed the efficacy of target drugs (P＜0.01).PAH target drugs significantly improved the one year survival rate of the severe cases.Conclusion The main clinical characteristics of SLE associated PAH include Raynand's phenomenon,pericardial effusion and positive anti-RNP antibody.The severity of PAH may not be related to lupus disease activity.PAH targeted drugs are effective in SLE-PAH.CTX may be effective in some cases.For severe cases,the combination therapy of CTX and PAH targeted drugs could significantly improve the prognosis.

ObjectiveTo evaluate the clinical and radiological efficacy of TNFR Ⅱ -Fc combined with methotrexate( MTX ) in treatment of patients with moderate and severe rheumatoid arthritis.MethodsThree hundred and ninty-six RA patients were randomized into the combined treatment group,the TNFR Ⅱ -Fc only group and MTX only group.All patients were treated for 24 weeks.ACR-N,ACR20,ACR50,ACR70,DAS28-ESR and Sharp score of both hands were measured for efficacy,and the side-effects were analyzed by one-way ANOVA.Results After 24-week therapy,the ACR-N of the combined treatment group [( 12.79±9.24)％-year] was significantly improved than that of the TNFR Ⅱ-Fc only group [(9.56±11.16)％-year,P＜0.05] and that of the MTX only group[(5.08±11.10)％-year,P＜0.05],and the TNFR Ⅱ-Fc group was significantly improved than that of the MTX group(P＜0.05).The ACR20 response rate of the combined group(80.4％) was significantly higher than that of the TNFR Ⅱ -Fc group(71.1％,P＜0.05) and the MTX group(56.7％,P＜0.01 ).The ACRS0 response rate of the combined group(53.6％) was significantly higher than that of the MTX group(30.8％,P＜0.01 ).The ACR70 response rate of the combined group was 27.7％,which was significantly different from that of the TNFR Ⅱ -Fc group (15.8％) and MTX group (7.7％,P＜0.05or P＜0.01 ).DAS28-ESR in the combination group was significantly reduced than those of the TNFR Ⅱ -Fc group and MTX group,and the DAS28-ESR of the TNFR Ⅱ -Fc group was significantly reduced than MTX group.The average total Sharp score of both hands,which demonstrated the radiographic changes,was significantly reduced in the combination group than the MTX group(P=0.03).The total adverse events in the combined group(40.9％) was significantly high than that of the MTX group(28.8％,P＜0.05).Conclusion TNFR Ⅱ -Fc combined with MTX can effectively control the activity of RA and radiological progress.

Objective To evaluate the prevalence of atherosclerosis in Chinese premenopausal women with systemic lupus erythematosus (SLE) and study possible associations between non-traditional risk factors with premature atherosclerosis. Methods One hundred and eleven premenopausal women with SLE and 40 healthy controls without clinical cardiovascular disease were evaluated. B-mode ultrasonography was used to measure carotid plaque and intima-media wall thickness( IMT). The relationship between the patients' clinical characteristics and carotid plaque was examined. At the same time, B-mode ultrasound was used to measure flow-mediated dilation (FMD) and nitroglycerin-mediated dilation (NMD) in the brachial artery. Using this method, the difference in endothelial function between SLE patients and controls was assessed. T-test,χ2 test and logistic regression were used for statistical analysis. Results Carotid plaque was more frequently observed in patients with SLE (16 of 111 patients) than in control subjects (0 of 40 subjects) (P=0.007). The mean IMT (m-IMT) (0.62 mm vs 0.45 mm, P<0.01) and maximum IMT(M-IMT) (0.7 mm vs 0.6 mm, P<0.01) was significantly higher in patients than in controls. As compared with patients without plaque, patients with plaque were significantly older, had longer disease duration, higher body mass index (BMI), higher blood pressure, shorter prothrombin time, elevated C-reactive protein level, higher SLICC score, higher cumulative prednisone dose, less hydroxychloroquine accumulated dosage, higher m-IMT and M-IMT, lower FMD and NMD. In logistic regression analysis, older age (P=0.012, OR=1.137), higher BMI (P=0.051, OR=1.205) and higher SLICC score (P=0.000, OR=2.888) were independently related to the presence of plaque. Conclusion SLE patients have higher prevalence of carotid atherosclerosis plaque than healthy controls and the age at onset is younger than controls. In addition to traditional risk factors for cardiovascular disease, SLE itself and disease related factors play important roles in premature atherosclerosis in SLE. SLE patients have significant endothelial dysfunction. Thus, endothelial dysfunction can be regarded as one manifestation of premature atherosclerosis in SLE.

Objective To investigate the mRNA expression of IKB kinase (IKK-α) and interferon-α (IFN-α) in the peripheral blood leukocytes of patients with systemic lupus erythematosus (SLE), and to explore the role of IKK-α in the production of IFN-α in SLE patients. Methods SYBR green dye I based real-time quantitative PCR was used to detect the mRNA expression levels of IKK-α and IFN-α in the peripheral blood leucocytes of SLE patients and healthy controls. Serum levels of IFN-α were measured with ELISA method. Results IKK-α mRNA expression levels in SLE patients were significantly higher than those of normal controls (P<0.05). IKK-α mRNA expression levels in SLE patients with active disease were significantly higher than patients with stable disease (P<0.01). IFN-α mRNA expression level in SLE patients was significantly lower than that of the normal controls (P<0.01). IFN-α mRNA expression levels in SLE patients with active disease were significantly higher than patients with stable disease (P<0.01). Serum levels of IFN-α in SLE patients with active disease was significantly higher than that of the normal controls and patients with stable disease (P<0.05). The anti-dsDNA antibody correlated positively, and complement C3 correlated negatively with serum concentration of IFN-α. IKK-α mRNA expression levels in SLE patients correlated positively with serum concentration of IFN-α. Conclusion IKK-α correlates positively with serum concentration of IFN-α. The IFN-α level is significantly correlated with disease activity, This suggests that IKK-α may play an important role in the pathogenesis of SLE.

Objective To study the incidence of osteopenia in patients with initial systemic lupus erythematosus(SLE). Investigate the levels of the vitamin D (VitD) endocrine system in peripheral blood of SLE patients and its relation to bone mineral density (BMD). Analyse the relationship between the estrogen receptor (ER) and BMD and evaluate the role of ER in the pathogenesis osteopenia. Methods Serum levels of 25-OH VitD_3 and 1,25-(OH)_2 VitD_3 were detected by enzyme linked immunosorbent assay. The gene expression levels of VitD receptor (VDR) and ER were determined by real-time PCR. BMD measurements in the lumbar spine (L1-L4) and left proximal femur (femoral neck) were performed using dual X-ray absorptiometry before treatment. Results The initial SLE patients had significantly lower BMD values, and higher frequency of bone loss at both sites of measurement compared with normal controls (P < 0. 05). The levels of 25-OH VitD_3 and 1,25-(OH)_2 VitD3 were lower in the initial SLE patients than normal controls(P<0.01 both). There is no difference in the levels of 25-OH VitD_3 and 1,25-(OH)_2 VitD_3 between the osteopenia SLE group and the normal BMD SLE group (P > 0. 05, P > 0. 05). There are no correlations between the Vitd and BMD in initial SLE patients (P>0.05 both). The expressions of VDR gene were significantly increased in the initial SLE patients compared with the normal controls(P<0.01). There was no difference in VDR gene expression between osteopenia SLE group and normal BMD SLE group (P>0.05). The VDR gene expression does not correlate with the bone mass (P>0.05). The levels of ER-β gene expression are higher in the initial SLE group than the normal controls (P<0.01).Conclusions The incipient SLE patients may have lower BMD than expected. SLE patients present abnormal VitD endocrine system and higher ER-β mRNA expression than those in normal controls, but these weren't concerned with osteopenia.

Objective ① To investigate the level of the vitamin D endocrine system in peripheral relationships with bone mineral density (BMD) and the disease activity respectively. Methods The level of the 25-hydroxylate vitamin D3 (25OHD3) and 1,25-dihydroxyvitamin D3 [1,25(OH)D3] in plasma from 43 SLE patients and 44 normal controls were detected by enzyme linked immunosorbent assay. Vitamin D receptor (VDR) gene expression was determinied by real-time PCR in peripheral blood. BMD measurements in the lumbar spine (L1-4) and left proximal femur (femoral neck) were performed using dual X-ray absorptiometry before treatment. The relationship between the vitamin D endocrine system and the bone mass were studied. We also discussed the relationship between the vitamin D endocrine system and the disease activity. Results The levels of 25OHD3 and 1,25 (OH)2D3 were lower in the initial SLE patients than normal controls (P<0.01, P<0.01). The expressions of VDR gene were significantly increased in initial SLE compared with normal controls (P<0.01). The initial SLE patients had significantly lower BMD values, and higher frequency of osteopenia (35%) at both sites of measurement compared with matched healthy controls (P<0.01). The initial SLE patients were divided into two groups by BMD, abnormal group and normal group. There were no differences in 25OHD3, 1,25 (OH)D3 and VDR gene expression (P0.05). There was no correlation between the vitamin D endocrine system and BMD in initial SLE patients. There was no correlation between the vitamin D endocrine system and the disease activity either. Conclusion Vitamin D endocrine system may play an important role in SLE, but the level of VDR gene is not correlated with BMD and disease activity.

Objective To analyze the clinical features of infective endocarditis with positive antineutrophil cytoplasmic antibodies ,and compare with ANCA associated small vessel vasculitis(AASV). Methods Three IE patients with positive ANCA were analyzed, and 13 cases from literatures were reviewed. Results Sixteen patients had positive anti-PR3 ANCA, in which 2 cases had both positive (anti-PR3 and anti-MPO ANCA) ANCA. All patients had some clinical manifestations mimic AASV, including fever ( 13/16, 81% ), rash (8/16, 50% ), rapidly progressive glomerulonephritis (7/16, 44% ), splenomegaly (6/16, 38% ). Streptococcal species were identified in 12 patients, and cardiac valvular abnormalities were demonstrated in all patients. All patients except 2, who died of cerebral hemorrhage followed by cerebral infarction, recovered with antibiotic therapy. Conclusion Infective endocarditis sometimes can have the same clinical features as AASV, so physicians should carefully differentiate between them when dealing with patients with positive ANCA antibodies.

Objective To correlate the chemokine score with disease activity,organ damages and clinical features in systemic lupus erythematosus (SLE) patients.Methods Peripheral blood cells obtained from 60 SLE patients,20 rheumatoid arthritis (RA) patients and 23 healthy donors (HDs) were subjected to real-time PCR to measure the transcriptional levels of seven chemokines (RANTES,MCP-1,CCL19,MIG,IP-10,CXCL11,and IL-8).Chemokine scores were calculated and were compared between various groups of SLE patients as well as between patients and controls.Results Chemokine scores were significantly elevated in SLE patients compared with RA patients and HDs (P=0.0112 and P=0.0019,respectively).Chemokine scores were correlated positively with SLEDAI (P=0.0061) and negatively with C3 levels (P=0.003).Compared to patients without lupus nephritis (LN),chemokine scores were elevated in SLE patients with active LN,especially when their daily prednisone dosage was less than 30 mg (P=0.0418 and P=0.002,respectively).Chemokine scores were also associated with cumulative organ damage (SLICC damage index [SDI]＞0) and positive anti-Sm and anti-RNP autoantibodies.Conclusion The chemokine score may serve as a new biomarker for disease activity and organ damage in SLE patients.

Objective To evaluate the efficacy and safety profile of a recombinant human tumor necrosis factor receptor: Fc fusion protein in ankylosing spondylitis (AS). Methods This was a multicenter,randomized, double-blind, placebo-controlled trial in the first 6 weeks and then followed by an open-labeled trial in the next 6 weeks. One hundred and forty-three patients of active AS were randomly assigned to receive 25 mg twice-weekly subcutaneous injections of rhTNFR:Fc or placebo for 6 weeks. The primary endpoint was proportion of ASAS20 responders at week 6. The secondary endpoints were the proportion of subjects achieving a BASDAI 20%, BASDAI 50% and BASDAI 70% improvement at week 6. Other secondary endpoints, related to reducing signs and symptoms of AS and improving range of motion and physical function, were evaluated.Results Treatment with rhTNFR:Fc resulted in significant improvement. At 6 weeks, 68% of the 71 patients in the rhTNFR: Fc group had a treatment response, as compared with 28% of those in the placebo group(P＜0.01). Improvements over base-line values for other measures of disease activity were significantly greater in the rhTNFR:Fc group, rhTNFR:Fc was well tolerated, The most frequently treatment related adverse event was injection site reaction. Conclusion rhTNFR:Fc has demonstrated consistent evidence of efficacy and is well tolerated in the treatment of active AS.