Objective:To describe the clinical features and immunological of Uygur systemic lupus erythematosus (SLE) subjects.Methods:Atotal of 484 adult patients (≥18 years) followed in the People's Hospital of the Xinjiang Uygur Autonomous Region were included into this study including 211 Uygur patients with SLE and 273 Han patients with SLE.Results:① Of the 211 Uygur SLE patients, 195 (92.4%) were female and 16(7.6%) were male (female:male=12.2∶1), the mean age at SLE onset was 15-64(35±12) years, mean disease duration was 0.1-228(21±35) months.② Of the 211 Uygur SLE patients, the most prevalent clinical presentationswere renal involvement 56.4%(119/211), hematological 56.4%(119/211), rash 50.2% (106/211), arthralgia/arthritis 49.8%(105/211), weakness 47.9%(101/211). The significant difference observed between the Uygur and Han SLE patients was Raynaud phenomenon( χ2=6.066, P=0.014) .③ The positive rates of antinuclear antibodies were 94.8%(200/211), 54.5%(115/211) for anti-dsDNA antibodies, 47.9% (101/211) for anti-SSA antibodies, 35.1%(74/211) for anti-U 1-RNP antibodies, and 28.4%(60/211) for anti-AHA antibodies. The positivity of anti-AHA antibodies ( χ2=4.952, P=0.026) was higher in Uygur SLE patients than thatin Han SLE patients. More Uygur SLE patients were anemic ( χ2=6.904, P=0.009), with decrease of complement ( χ2=6.330, P=0.012). Conclusion:This study can not find any significant differences between the Uygur SLE patients and Han SLE patients in common clinical manifestations in Xinjiang. The Uygur SLE patients are more likely to have anemia and decrease of complement, which may be related to race and diet habits.

Objective To assess the clinical characteristics of systemic lupus erythematosus (SLE) in the elderly in Xinjiang.Methods As a retrospective study,clinical date and laboratory test results were collected from 87 elderly SLE patients and 222 non-elderly SLE patients (age ＜ 50 years) hospitalized in the People's Hospital of the Xinjiang Uygur Autonomous Region from January 2006 to July 2016.Results The female to maleratio was 5.7 ∶ 1.0 in the older-onset SLE group and the female to male ratio was 11.3 ∶ 1.0 in the younger-onset SLE group.Of the 13 patients with predisposing factors in the older-onset SLE group(14.9 ％),11 had infection (12.6 ％),whereas the 27 patients with predisposing factors in the younger-onset SLE group (12.2 ％)had pregnancy,abortion or insolation,in addition to infection.Arthritis (63.2 ％),weakness (40.2 ％),malar rashes (36.8 ％),anorexia(26.4 ％),and shortness of breath(26.4 ％) were common clinical manifestations in the olderonset SLE group.The incidence of anorexia in the older-onset SLE group was higher than that in the younger-onset SLE group(P＜0.01),while the incidence of trichomadesis was lower in the older-onset SLE group than that in the younger-onset SLE group(P＜0.01);Aminotransferase elevation,creatinine elevation and thrombocytopenia were more common in the older-onset SLE group than in the younger SLE group (P＜0.05).Lower rates of positive anti-Smanti body and anti-Acl antibody were found in the older onset SLE group,compared with the younger-onset SLE group (P＜0.05);Han older-onset SLE patients showed higher rates of oral ulcers than older-onset SLE patients of Uygur,Kazak or other ethnic minorities (P＜0.05).There were no significant differences between Han and Uygur/Kazak patients in laboratory test results.Conclusions The proportion of male SLE patients in the elderly is higher than that in non-elderly SLE patients in Xinjiang.Also,elderly SLE patients are prone to oral ulcers but often do not show typical early clinical symptoms and have low levels of specific antibodies.Therefore,clinicians should pay particular attention to older onset SLE patients in order to reduce the rates of misdiagnosis and missed diagnosis.

Objective To discuss the characteristics of in-patients with Beh(c)et's disease in Xinjiang Uygur Autonomous region.Methods Clinical data of 146 cases with Behcet's disease admitted to Xinjiang Uygur Autonomous region people's hospital were analyzed.The demographic data and clinical manifestations were analyzed retrospectively.The categorical data was compared using x2 test or Fisher's exact test.Results ① Of the 146 patients with BD,99 cases were Han,47 cases were uyghurs.Oral ulcer were observed in 146 cases (100％).Genital ulcer occurred in 115 cases (78.8％),of which 76 were Han (76.8％),39 (82.9％) were uyghurs,the difference was not significant (P=0.391).The initial presentation was oral ulcer in 83 cases (56.8％),of which 60 cases (60.6％) were Han,23 cases (48.9％) were the uyghurs.The second most common presentation was oral ulcer and genital ulcer,which occurred in 42 cases (28.8％),of which 23 cases (23.2％) were Han,19 cases (40.4％) were the uyghurs.More Han patients initially presented with both oral and genital ulcers than uyghur (P=0.032).② Skin lesions were recorded in 66 cases (45.2％),of which 45 cases (45.4％) were Han,21 cases (44.7％) were the uyghurs,the difference was not significant (P=0.930).③ Joint damage occurred in 40 cases (27.4％),of which 22 cases (22.2％) were Han,18 cases (38.3％) were the uyghurs.Joint damage wasmore frequently observed in Han than in uyghur (P=0.042).④ Eye lesion happened in 21 cases (14.4％),ofwhich 9 cases (9.1％) were Han,12 cases (25.5％) were the uyghurs,ie,eye lesion of Uyghur was higher than Han (P=0.008).⑤ Vascular lesions were detected in 8 cases (5.5％),of which the 5 cases were Han,3 cases were uyghurs,the difference was not significant (P=0.712).⑥ Nervous lesions were observed in 2 cases (1.37％),1 was Han and one was uyghur,the difference was not significant (P=0.542).Conclusion The most common clinical presentations of BD in Xinjiang region are oral and genitalulcers.Skin,joints,eye,vascular,nervous system can also be affected.Joint damage in Han is higher than Uyghur,but eye lesion of Uyghur is more common than Han.

Objective:To explore the depression of patients with systemic lupus erythematosus (SLE) during the coronavirus disease 2019 (COVID-19) pandemic.Methods:Patients with SLE and healthy subjects(control group) from August 16, 2020 to September 2, 2020 in Urumqi, Xinjiang were collected. The depression and sleep quality were assessed by Self rating Depression Scale (SDS) and Pittsburgh sleep quality index (PSQI). The nonparametric data were represented by median (quartile spacing), and the rank sum test was used to compare the continuous variables between the two groups. Pearson correlation test was used for continuous variables, and Spearman correlation test was used for rank variables or classified variables. Statistical product and service solutions.Results:The prevalence rate of depression in the SLE group (41.9%) was significantly higher than that in healthy control group (32.1%). The difference was statistically significant ( χ2= 10.312, P<0.01). The prevalence rate of poor sleep in the SLE group (31.8%) was significantly higher than that in healthy control group (21.6%). The difference was statistically significant( χ2=3.885, P=0.049). The total score of VAS ( OR=1.366, P<0.01) , PSQI( OR=1.198, P<0.01) were the risk factor of depression in patients with SLE. Conclusion:SLE patients have a higher prevalence of depression during pandemic quarantinee-period, pain and poor sleep quality are risk factors. We need to pay attention to the psychological status of SLE patients.

Objective: To assess the association between lupus nephritis disease activity and anti-C1q antibodies. Methods: The study analyzed the medical records of 98 patients with lupus nephritis (LN), 35 patients without lupus nephritis. LN disease activity was measured by the systemic lupus international collaborating clinics (SLICC) renal activity score of 2008. All biopsied tissues were scored based on the International society of nephrology/Renal pathology society (ISN/RPS) 2003 LN pathological typing standards, acute and chronic index scores were used to evaluate the activities of lupus. All patients were test for the levels of anti-dsDNA and anti-C1q antibodies using the enzyme-linked immuno sorbent assay (ELISA), C3, C4, 24-hour urinary protein performed in parallel. For normally distributed quantitative parameters, the differences between groups were assessed by t test. Mann-Whitney U test was performed for non-normally distributed data. The cut-off values were evaluated by using receiver operating characteristic (ROC). The Spearman methods were used to test correlations. Results: Patients with LN had a higher levels of anti-C1q antibodies than patients without lupus nephritis [3.94 (10.2, 91.3) AU/ml与6.9 (2.0, 15.4) AU/ml; Z=-4.299, P<0.01]. Patients with inactive lupus nephritis had higher levels of C1q, C3, C4 than active LN (t=2.393, 3.777, 2.557; P<0.05). Patients with active lupus nephritis had higher levels of anti-C1q antibodies than inactive LN (Z=-4.632, P<0.01). Anti-C1q antibody levels were positively correlated with levels of 24-hour urinary protein, AI score (r=0.327, P<0.01) and SLICC score (r=0.493, P<0.01), and were negatively correlated with serum C1q (r=-0.373, P<0.01), C3 (r=-0.532, P<0.01) and C4 (r=-0.463, P<0.01). The optimal cutoff value of Anti-C1q for a diagnosis of active LN was 48.9 RU/ml, and the sensitivity and specificity were 62.5% and 80%, respectively. The area under the curve (AUC) was 0.771. Conclusion Anti-C1q antibodies are more closely correlated with renal disease activity, and anti-C1q antibody is an important serum marker for monitoring LN activity, but its pathological mechanism in the pathogenesis of LN still needs to be further explored.

Objective:To investigate the risk factors of uveitis in ankylosing spondylitis (AS).Methods:This retrospective study included 206 patients with AS who visited the department of rheumatology and immunology of People's Hospital of Xinjiang Uygur Autonomous Region between January, 2018 and December, 2018. Those patients with uveitis were enrolled in the uveitis group. AS patients without uveitis were included in the non uveitis group as control. The basic clinical data, laboratory indexes and imaging data were analyzed retrospectively by t-test, Wilcoxon rank sum test, Chi square test and binary logistic regression. Results:Thirty-seven patients with uveitis and 169 patients without uveitis were included. Compared with the non uveitis group, patients with uveitis were older in age [(40±11) years vs (36±11) years, t=-2.06, P<0.05], longer in disease duration [10(5, 16) years vs 5(2, 10) years, Z=-3.16, P<0.01], more peripheral arthritis [51.4%(19/37) vs 32.5%(55/169), χ2=4.66, P<0.05] and peripheral enthesitis [40.5%(15/37) vs 11.8%(20/169), χ2=17.34, P<0.01], and higher human leukocyte antigen (HLA)-B27 positive rate [100%(37/37) vs 85.8%(145/169), χ2=5.95, P=0.01]. However, there were no significant difference in gender, race, cervical tenderness, smoking history and volume, a positive family history of uveitis, a positive family history of AS and BMI. There was no significant difference in bloodplatelet (PLT), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR). By the binary logistic regression analysis, we found that peripheral enthesitis [ OR(95% CI)=4.289(1.832, 10.040), P<0.01], and longer disease duration [ OR(95% CI)=1.072(1.014, 1.134), P<0.05] were independently related to AS related uveitis. Conclusion:This study suggests that the risk of uveitis is increased in AS patients with longer disease duration and peripheral enthesitis.

Objective:To evaluate the efficacy and safety of belimumab in the treatment of systemic lupus erythematosus (SLE).Methods:Retrospective analysis was made in 41 patients with SLE who were treated with belimumab in our hospital. The demographic data, clinical features, laboratory test results and treatment-related adverse events (AE) were collected and analyzed. Paired sample t test and Wilcoxon test were used to compare and analyze the changes of laboratory indexes before and after treatment. Results:The follow-up time of the 41 patients was (3.0±1.0) months, and 19 patients stopped belimumab because of the Covid-19 pandemic, the duration for belimumab withdrawal was (2.6±0.9) months. The most common clinical manifestation was impaired renal function (63.4%, 26 cases), followed by musculoskeletal impairment (58.5%, 24 cases). Before and after treatment, the dose of glucocorticoid was significantly decreased [15(9, 35) mg/d vs 13(8, 25) mg/d, Z=-3.573, P<0.01], the systemic lupus erythematosus disease activity index (SLEDAI)-2000 was significantly decreased [5(4, 8) vs 2(2, 4), Z=-4.446, P<0.01], the anti-double-stranded DNA (dsDNA) antibody [enzyme-linked immune-sorbent assay (ELISA)] level was significantly decreased [96(26, 300) vs 36(10, 139), Z=-3.436, P<0.01], the complement C3 level was significantly increased [(0.62±0.22) g/L vs (0.74±0.20) g/L, t=-5.454, P<0.01], the complement C4 level was significantly increased [(0.13±0.07) g/L vs (0.17±0.07) g/L, t=-5.851, P<0.01), the hemoglobin level was significantly increased [(117±15) g/L vs (125±16) g/L, t=-4.236, P<0.01) and A/G level was significantly increased [(1.30±1.36) vs (1.49±0.29), t=-5.174, P<0.01]. Four patients (13.8%) had adverse events during treatment with belimumab, including 1 case of upper respiratory tract infection, 2 cases of urinary tract bacterial infections and 1 case of herpes zoster. Conclusion:Belimumab is safe and effective in the treatment of SLE. It can significantly reduce the dosage of glucocorticoid and improve anemia, but the specific mechanism needs to be further studied.

Objective:To investigate the efficacy and safety of tofacitinib (TOF) in the treatment of psoriatic arthritis (PsA).Methods:The clinical data of 5 patients with PsA from September 2018 to December 2020 in People's Hospital of Xinjiang Uygur Autonomous Region were collected. Five patients were treated with a variety of disease-modifying anti-rheumatic drugs (DMARDs), two of them had ever been treated with biologic disease-modifying antirheumatic drugs (bDMARDs) [recombinant human tumor necrosis factor-alphareceptor Ⅱ: IgG Fc (rhTNFR: Fc, Adalimumab], but failed to show efficacy or relapse after drug withdrawal. Multiple joints were involved in 2 patients. These five patients were treated with tofacitinib. Their data were collected and analyzed 3-month and 6-month after treatment respectively, including the changes of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), severity of pain measured by visual analogue scale (VAS), joint pain count (TCJ), joint swelling count (SCJ), health assessment questionnaire (HAQ), 28 joint disease activity score (DAS28 CRP), psoriasis area and severity index (PASI), and PsA disease activity index (DAPSA). Adverse reactions were observed and analyzed.Results:These 5 cases were treated with TOF 5 mg twice daily. Three months after treatment, swelling joints count and psoriatic rash were significantly improved, and pain was significantly relieved in 4 cases. Six months after treatment, the ESR, CRP, VAS, TCJ, SCJ, HAQ, DAS28 CRP, PASI, and DAPSA decreased further. According to DSA28-CRP score, peri-pheral joints involvement of 3 cases were improved, and 2 cases reached low disease activity state. The overall effective of PASI were observed in 4 cases. According to the DAPSA score, 1 case reached the PsA disease remission state and 4 cases reached the PsA low disease activity state. No remarkable adverse reactions occurred.Conclusion:With good therapeutic effect and less adverse reactions, TOF is a potential treatment option for PsA.

Objective To explore the correlation between chemokine C-C motif ligand 2 (CCL2)-2518A/G polymorphism and the incidence of systemic lupus erythematosus (SLE),and investigate the effects of CCL2-2518A/G polymorphism on the expression of CCL2.Methods A total of 134 SLE patients and 56 sex and age matched healthy people were enrolled in this study.CCL2 plasma levels were measured by enzymelinked immunosorbent assay(ELISA).The 2518 A/G polymorphism in CCL2 promoter region was genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP),and then peripheral blood mononuclear cells (PBMCs) were cultured in vitro to explore the influence of the 2518A/G polymorphism in CCL2 promoter region on the expression of CCL2.Mann-Whitney U-test,Student's t test and chi-squared test were used for analysis.Results The plasma CCL2 level in the SLE group was 358.5 (500.1) pg/ml [median (four interval)],which was significantly higher than that in the control group 243.6(125.8) pg/ml (Z=3.892,P=0.000).Patients with high plasma CCL2 levels were more prone to have renal (x2=7.159,P=0.007) and der-matomucosal (x2=5.133,P=0.023) involvement,as well as much higher disease activity index (SLEDAI) scores (Z=2.012,P=0.047).The results of the analysis of individual CCL2-2518A/G polymorphic genotypes suggested that patients with the G/G genotype had the highest CCL2 levels 425.7 (608.8) pg/ml,followed by those with the G/A genotype 355.3(511.1) pg/ml and A/A genotype 327.8(367.9) pg/ml (x2=3.496,P=0.048).The results of in vitro experiment showed that after the stimulation of lipopolysaccharide,the expression of CCL2 in PBMCs with G/G homozygote increased more significantly than that with A/A homozygote (P＜0.05).Conclusion The increase of plasma CCL2 concentrations is associated with tissue injury and high SLEDAI,which suggests that CCL2 may play a crucial role in the pathogenesis of SLE.CCL2-2518A/G polymorphism is not related to the pathogenesis of SLE,but it can affect the condition of SLE by promoting the expression of CCL2 in inflammatory environment.

Objective:To analyze the 15-year survival rate, causes of death and prognostic factors of late-onset systemic lupus erythematosus(SLE).Methods:A total of 92 late-onset SLE patients aged >50 years hospitalized in the Rheumatology and Immunology Department of the People's Hospital of Xinjiang Autonomous Region from January 2006 to December 2016 were retrospectively collected.Clinical and serological data of the patients were analyzed and the diagnosis met the SLE classification criteria as revised by the American Society of Rheumatology in 1997.Survival rate, the cause of death, and the associated prognostic factors were analyzed.Outpatient or telephone follow-up was performed.Results:Of the 92 study patients, 8 were lost to follow-up(8 / 92, 8.7%)and 84 were able to follow up(84 / 92, 91.3%). Of the 84 able to follow up, 64 cases(64 / 84, 76.2%)survived, and 20 cases(20 / 84, 23.8%)died.Median survival time was 108 months.The 5-, 10-, and15-year survival rates were 86.0%, 77.9%and 59.6 %, respectively.Infection(55.0%)and multiple organ failure(15.0%)were the major cause of death in patients with late-onset SLE.Univariate analysis of the outcomes of patients with late-onset SLE showed that the age of onset( HR=1.113, P=0.001), hematology involvement( HR=3.610, P=0.023), and complicaed cardiovascular disease( HR=3.128, P=0.011)had a significant impact on the prognosis. Conclusions:The late-onset SLE has low survival rate and bad prognosis.Infection prevention and control of organ damage and focus on merging cardiovascular disease are critical to improve survival of late-onset SLE patients.