OBJECTIVE:The aim of this study was to establish a fast, practical and automated assay to measure the expression of interferon-stimulated genes (ISGs) in whole peripheral blood and evaluate the utility of these markers in distinguishing active SLE. METHODS:A total of 102 SLE patients (38 active, 64 inactive) were enrolled. The mRNA levels of five ISGs (IFI27, OAS1, IFI44L, LY6E, IFIT1) in whole blood were quantified using an automated multiplex RT-qPCR platform. Multivariable linear regression was performed to evaluate the influence of medications and specific autoantibodies on ISG expression. Diagnostic performance was evaluated via receiver operating characteristic curves. Backward stepwise logistic regression was employed to identify optimal predictors and subsequently construct a diagnostic probability matrix for disease activity. RESULTS:ISGs expression were significantly elevated in active SLE patients (2.9-17.3 fold) and positively correlated with disease activity indictors. Multivariable analysis confirmed that ISGs expression reflected disease activity independent of treatments and autoantibody status. Subsequently, IFI27 and OAS1 were identified as the optimal combination, forming the ISG2 Score. Multivariable analysis confirmed that the ISG2 Score and complement-3 (C3) were independent predictors of active SLE. An ISG2-C3 matrix was developed and revealed a dramatic stepwise escalation in disease probability. Notably, we observed that elevated IFI27 in clinically inactive patients was associated with persistent leukopoenia. CONCLUSION:This automated whole-blood assay offers a rapid and reliable method for ISGs detection. IFI27 and OAS1 combined with C3, serve as sensitive biomarkers for monitoring of active SLE in routine care.

ObjectiveTo develop a guideline for selecting biomarkers in the diagnosis and assessment in patients with axial spondyloarthritis (axSpA).MethodA joint effort was carried out by the core team, the literature review team and the multidisciplinary voting panel to formulate recommendations regarding biomarkers in axSpA, using an evidence-based and consensus-based strategy. Certainty of evidence and strength of recommendation were determined, and levels of agreement within the voting panel were calculated.ResultsA total of 20 recommendations were formulated in this guideline, with levels of agreement ranging from 6.48 to 9.71. The two strong recommendations, HLA-B27 testing in patients suspected of axSpA and regular-interval monitoring of CRP/ESR represent the status quo of axSpA evaluation, while the 13 conditional recommendations represent the promising biomarkers with clinical utility in diagnosis, disease activity assessment, prediction of radiographic progression and therapeutic responses. This guideline does not dictate clinical choices of tests on axSpA, and decisions should be made based on comprehensive consideration of costs, accessibility, patients’ values and willingness as well as the objective of testing in the local context.ConclusionThis guideline addresses the interpretation of the clinical significance of biomarkers in axSpA, and the biomarkers endorsed in this guideline composed a clinical toolkit for healthcare professionals to choose from.

本研究从学生视角解析课堂评价行为,考虑中小学的评价情境,形成问卷测试条目.对678名中小学生进行问卷数据的项目分析和探索性因素分析,对652名中小学生进行问卷数据的验证性因素分析,结果发现:课堂评价行为问卷是一个三因子的整体结构,分别是教师的课堂评价行为、学生自我评价行为、学生同伴评价行为,共计37个条目.通过对正式问卷的内部一致性信度、分半信度、内容与结构效度检验表明,该问卷具有较高的信效度,可用于测评小学四年级至高三年级学生的课堂评价行为状况.抽样某县16758个中小学生进行正式问卷的调查,结果发现:课堂评价行为总体水平中等偏上,但内部存在差异,学生的自我评价和同伴评价明显低于教师的课堂评价,而且在性别、学段、城乡和成绩上存在显著差异.我们需要正视课堂评价的整体性,审视课堂评价行为水平的差异性,重视学生特征对课堂评价行为的影响.

Objective:To explore the efficacy and safety of low-dose rasburicase for refractory chronic gouty arthritis.Methods:A cohort study. The clinical data of patients with refractory chronic gouty arthritis who were treated with rasburicase at Sun Yat-sen Memorial Hospital, Sun Yat-sen University between January 2021 and July 2022 were retrospectively analyzed. Refractory chronic gouty arthritis was defined as serum uric acid (sUA)>360 μmol/L and urate volume>10 cm 3 under dual-energy computed tomography after tolerable maximal oral urate-lowering therapy for at least 3 months. The administration of low-dose rasburicase was applied intravenously with total dosage ranging from 4.5 to 7.5 mg each dose, at 4-week intervals for a maximum of three doses. Efficacy was evaluated by the changes of sUA level, tophus and urate volume. Results:A total of 22 patients were included for analysis, with 95.4% (21/22) male, the mean age was (44±15) years, and the median duration of gout was 11 (6-15) years. The mean sUA at baseline was (667±112) μmol/L. The levels of sUA significantly decreased after each dose of rasburicase ( P<0.001), and the median reduction of sUA after each dose of rasburicase was 568 (471-635), 187 (66-335) and 123 (49-207) μmol/L, respectively. At week 12, nine patients (40.9%) exhibited sUA<360 μmol/L and tophus disappeared in one patient. The urate volume significantly decreased at week 12 when compared with that before the first dose of rasburicase in all the patients [40 (16-172) cm 3 vs 17 (7-134) cm 3, P<0.001], with a median reduction rate of 41.6% (22.9%-58.5%). The everall safety of rasburicase was good, and no serious adverse reactions occurred. Conclusions:Low-dose rasburicase is well-tolerated and effective for decreasing the urate burden in patients with refractory chronic gouty arthritis. Further prospective randomized controlled trials are needed to validate these findings.

OBJECTIVE:To explore the clinical and genetic characteristics of a Chinese pedigree affected by glycogen storage disease (GSD) type Ia with gout as the first manifestation.METHODS:Clinical and biochemical data of the pedigree were collected. Available members of the pedigree were subjected to gene sequencing, and the result was analyzed by bioinformatics software. The pedigree was followed up for five years.RESULTS:The proband was a young female manifesting recurrent gout flare, hypoglycemia, and hypertriglyceridemia. One of her younger brothers also presented with dysplasia and hepatic adenoma. Gene sequencing revealed that the proband and her younger brother both harbored c.1022T>A (p.I1e341Asn) and c.230+5G>A compound heterozygous variants of the G6PC gene , which were inherited from their father and mother, respectively. Among these, the c.230+5G>A is an intron region variant which was unreported previously, and bioinformatics analysis showed that it may impact mRNA splicing of the gene. The proband was treated with raw corn starch, allopurinol, and fenofibrate. Gout was well controlled, and she had given birth to a baby girl without GSD.CONCLUSION:GSD Ia should be considered among young gout patients with hypoglycemia and hepatomegaly, for which gene sequencing is warranted. GSD Ia has a good prognosis after comprehensive treatment with diet and medicine.

ObjectivesThe purpose of this study was to investigate the baseline independent risk factors for predicting 6-month mortality of patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis (DM) and develop a matrix prediction model formed by these risk factors.MethodsThe hospitalized patients with DM who completed at least 6-month follow-up were recruited as a derivation cohort. The primary exposure was defined as positive anti-MDA5 at the baseline. The primary outcome was all-cause 6-month mortality after enrollment. A matrix prediction model was developed in the derivation cohort, and another published cohort was used for external validation.ResultsIn derivation cohort, 82 patients with DM were enrolled (mean age of onset 50 ± 11 years and 63% women), with 40 (49%) showing positive anti-MDA5. Gottron sign/papules (OR: 5.135, 95%CI: 1.489–17.708), arthritis (OR: 5.184, 95%CI: 1.455–18.467), interstitial lung disease (OR: 7.034, 95%CI: 1.157–42.785), and higher level of C4 (OR: 1.010, 95%CI: 1.002–1.017) were the independent associators with positive anti-MDA5 in patients with DM. Patients with anti-MDA5-positive DM had significant higher 6-month all-cause mortality than those with anti-MDA5-negative (30 vs. 0%). Among the patients with anti-MDA5-positive DM, compared to the survivors, non-survivors had significantly advanced age of onset (59 ± 6 years vs. 46 ± 9 years), higher rates of fever (75 vs. 18%), positive carcinoma embryonic antigen (CEA, 75 vs. 14%), higher level of ferritin (median 2,858 ug/L vs. 619 ug/L, all p < 0.05). A stepwise multivariate Cox regression showed that ferritin ≥1,250 μg/L (HR: 10.4, 95%CI: 1.8–59.9), fever (HR: 11.2, 95%CI: 2.5–49.9), and positive CEA (HR: 5.2, 95%CI: 1.0–25.7) were the independent risk factors of 6-month mortality. A matrix prediction model was built to stratify patients with anti-MDA5-positive DM into different subgroups with various probabilities of 6-month mortality risk. In an external validation cohort, the observed 6-month all-cause mortality was 78% in high-risk group, 43% in moderate-risk group, and 25% in low-risk group, which shows good accuracy of the model.ConclusionBaseline characteristics such as fever, ferritin ≥1,250 μg/L, and positive CEA are the independent risk factors for 6-month all-cause mortality in patients with anti-MDA5-positive DM. A novel matrix prediction model composed of these three clinical indicators is first proposed to provide a chance for the exploration of individual treatment strategies in anti-MDA5-positive DM subgroups with various probabilities of mortality risk.

教师的评价素养是教师依据促进学习的评价理念开展学生评价所表现出来的关键能力和伦理品格,由促进学生学习的评价理念、技能与伦理三维立体构成.但现实中教师的评价素养不容乐观,主要存在三方面问题:第一,考试思维统领评价活动,学教评一体化理念尚未成为教师群体的共识;第二,五项技能不娴熟,教师的自我发展意识与行动力不强;第三,评价伦理基本失范,教师鲜少反思改善.改善此种状况的有效办法是以县(市区)为单位实施系统策略,破解影响策略实施质量的三个问题,即区域教育考试管理者的评价素养问题、以学生成绩评估教师业绩的"潜规则"问题以及教师的日常评价实践改进问题.

本研究从素养视角解析深度学习内涵与结构,考察中小学生的学习情境,形成问卷测试条目.对683名中小学生进行问卷数据的项目分析和探索性因素分析,对669名中小学生进行问卷数据的验证性因素分析,结果发现:中小学生深度学习问卷是一个三维度九指标结构,分别是深度投入(活力、奉献、专注)、深入认知(关联策略、整合策略、反思策略)、深层结果(理解与迁移能力、批判与创造能力、协作与沟通能力),共计37个条目.通过对正式问卷的内部一致性信度、分半信度、内容与结构效度检验,表明该问卷具有较高的信效度,可用于测评小学四年级至高中三年级学生的深度学习状况与水平.

本文报道1例从全身型幼年特发性关节炎发展到成人斯蒂尔病,在糖皮质激素、传统改善病情抗风湿药及生物制剂和小分子靶向药治疗下仍出现双腕、髋及膝关节滑膜炎及骨质破坏,右侧股骨头缺血坏死,并且反复左膝关节肿胀伴活动受限,最后诊断腘窝多发滑膜囊肿.经托珠单抗联合甲氨蝶呤、来氟米特及甲泼尼龙积极控制基础疾病、关节腔抽液及倍他米松注射等治疗后病情控制,左膝肿胀缓解.

Objective:To investigate the prevalence of hypertension and its associated factors in rheumatoid arthritis (RA) patients.Methods:Consecutive Chinese patients with RA were recruited from August 2015 to September 2019 at Department of Rheumatology, Sun Yat-sen Memorial Hospital. Demo-graphic data and clinical data were collected including indicators of disease activity, functional assessment and radiographic assessment, comorbidities and previous medications. Logistic regression analysis was used to evaluate the related factors of hypertension in RA patients.Results:There were 674 RA patients recruited with 82.3%(555/674) female and mean age (50±13) years. The prevalence rate of hypertension was 32.9% (222/674), followed by dyslipidemia (9.9%, n=67), type 2 diabetes (8.8%, n=59), hyperuricemia (8.5%, n=43), fatty liver disease (8.0%, n=54), cardiovascular disease (6.2%, n=42) and chronic kidney disease (3.3%, n=22). Compared with those without hypertension, RA patients with hypertension had advanced age with longstanding disease duration, higher disease activity indicators, worse joint destruction, and higher proportions of comorbidities. Multivariate logistic regression analysis showed that comorbidities including hyperuricemia [ OR=1.977, 95% CI(1.002, 3.900)], dyslipidemia [ OR=1.903, 95% CI(1.102, 3.288)] and fatty liver disease [ OR=2.335, 95% CI(1.278, 4.265)] were risk factors of hypertension after adjustment for age and gender. Conclusion:Hyperten-sion is the most common comorbidity in RA patients which is associated with comor-bidities including hyperuricemia, dyslipidemia and fatty liver disease. Detection and management of hyperten-sion and other cardiovascular disease related comorbidities in RA patients should be emphasized.

Background Recently, the autoantibody recognizing melanoma differentiation-associated gene 5 (anti-MDA5) is of the greatest concern as a specific autoantibody of dermatomyositis (DM), since it delineates a unique clinical phenotype of DM with a high risk of life-threatening lung complications. Considering routine clinical characteristics at baseline are still desired candidates for screening potential mortality predictors, in order to as early as possible stratify the mortality risk in anti-MDA5 positive DM patients before making therapeutic strategies. Objectives To investigate the baseline independent risk factors for predicting 6-month mortality of anti-MDA5-positive DM patients and develop a matrix prediction model formed by these risk factors. Methods This was a real-world prospective observational study. The hospitalized patients with DM were included if they fulfilled the criteria including: aged over 18 years old; diagnosed as having DM according to the criteria proposed by Bohan and Peter or the modified Sontheimer definitions; and positive anti-MDA5 which was determined by both line immunoassay testing and enzyme-linked immunosorbent testing. The primary outcome was all-cause 6-month mortality after enrolment. A matrix prediction model was built with the mortality risk probability. Results There were 82 DM patients enrolled (mean age of onset 50±11 years and 63% female), with 40 (49%) showing positive anti-MDA5. Gottron sign/papules (OR: 5.135, 95%CI: 1.489~17.708), arthritis (OR: 5.184, 95%CI: 1.455~18.467), interstitial lung disease (ILD, OR: 7.034, 95%CI: 1.157~42.785), and higher level of C4 (OR: 1.010, 95%CI: 1.002~1.017) were independent associators with positive anti-MDA5 in DM patients. Anti-MDA5-positive DM patients had significant higher 6-month all-cause mortality than those with anti-MDA5-negative (30% vs. 0%). Among anti-MDA5-positive DM patients, compared to the survivors, non-survivors had significantly advanced age of onset (59±6 years vs. 46±9 years), higher rates of fever (75% vs. 18%), positive carcinoma embryonic antigen (CEA, 75% vs. 14%), higher level of ferritin (median 2858 ug/L vs. 619 ug/L, all p<0.05). Multivariate COX regression showed ferritin≥1250 μg/L (HR: 10.4, 95%CI: 1.8~59.9), fever (HR: 11.2, 95%CI: 2.5~49.9), and positive CEA (HR: 5.2, 95%CI: 1.0~25.7) were independent risk factors of 6-month mortality. According to the matrix prediction model, anti-MDA5-positive DM patients could be stratified into three subgroups based on various probabilities of predicted mortality: (i) High-risk: eight patients with two of the above three features (including fever, serum ferritin≥1250 μg/L, and positive CEA) had high predicted mortality probability with 64%~85% (three red grids in Figure 1A), and the actual mortality was 75% (n=6) with 60%, 100%, and 100% respectively in three red grids (Figure 1B). Five patients with all of three features had extremely high predicted mortality probability with 97% (95%CI: 70%~100%, the dark red grid of Figure 1A), and the actual mortality was 100% in Figure 1B; (ii) Moderate-risk: nine patients with one of the above three features had moderate predicted mortality probability with 11%~29% (three yellow grids in Figure 1A), and the actual mortality was 11% (n=1) with 0%, 0%, and 17% respectively in three yellow grids (Figure 1B); (iii) Low-risk: eighteen patients with none of the above three features had low predicted mortality probability with 2% (95%CI: 0.2%~20%, the green grid in Figure 1A), and the actual mortality was 0% in the green grid (Figure 1B). Conclusion Baseline characteristics of fever, positive CEA, and ferritin≥1250 μg/L are risk factors for 6-month all-cause mortality in anti-MDA5-positive DM patients. A novel matrix prediction model composed of these three clinical indicators is firstly proposed to provide a chance for exploration of individual treatment strategies in anti-MDA5-positive DM subgroups with various probabilities of mortality risk. Disclosure of Interests None declared

Background: Although antinuclear antibodies (ANAs), anti-SSA and anti-Ro52, are present in immunoglobulin preparations, it is unknown whether intravenous immunoglobulin (IVIG) therapy influences the testing of serum autoantibodies in patients with connective tissue diseases (CTDs). The present study aimed to investigate the dynamic change over time of serum ANA-related autoantibodies in patients with CTDs receiving IVIG therapy. Methods: Serum ANA-related autoantibodies were monitored in two patients with CTD before IVIG therapy and at different times after therapy. These autoantibodies were tested in different batches of immunoglobulin preparations from seven pharmaceutical companies. Results: One patient developed a new ANA pattern (cytoplasmic dense fine speckled pattern, AC-19) just after IVIG therapy. Both patients developed de novo positivity for AMA-M2 and anti-SSA, but returned negative 1 month after IVIG therapy. The residual liquid in patients' immunoglobulin preparations showed positive ANAs with a high titer of AC-19 (1:640), a low titer of the nuclear fine speckled pattern (AC-4, 1:80), positive AMA-M2, and positive anti-SSA. ANA-related autoantibodies were tested in 16 batches of immunoglobulin preparations and all had positive ANAs with two patterns: AC-19 (1:640 or 1:320) and AC-4 (1:80). AMA-M2 and anti-SSA were positive in 100% of the batches. Conclusion: Our study highlights high-titer AMA-M2 autoantibodies in immunoglobulin preparations and suggests their transient transfer into a patient's circulation via IVIG therapy. To avoid incorrect clinical decisions based on postinfusion antibody titers, our data recommend retesting 1-2 months after high-dose IVIG immunomodulatory treatment.

目的:提高临床对SSc合并高血压、肾功能不全的鉴别诊断水平。方法:报道1例SSc合并高血压、肾功能不全患者的临床特点及诊治经过，并进行分析讨论。结果:中年女性，SSc病史5年，出现头痛、肉眼血尿10 d，伴血压升高，实验室检查血肌酐升高，肾小球源性血尿，蛋白尿，补体低，ANA、抗dsDNA抗体、核周型ANCA阳性，肾活检病理示LN（Ⅳ型），予激素冲击、环磷酰胺、贝利尤单抗及对症治疗后症状缓解，狼疮病情活动指标恢复正常。结论:SSc患者出现血压和肌酐升高，除考虑硬皮病肾危象外，应考虑有无其他疾病，必要时行肾脏穿刺活检病理检查以明确诊断，避免漏诊。

中小学生感知到的作业负担是指"学生在一定时间和范围内所承担的作业责任以及由此产生的身心感受"(郑东辉2017:15).作业责任主要通过学生完成一定数量作业所需要的时间来表征,身心感受则是学生做作业过程中产生的身体反应和心理体验.概括起来,作业负担由作业时间、生理负荷、心理负担三个相互关联的要素构成.

Objective: To investigate the expression of peroxisome proliferator-activated receptor-gamma coactivator (PGC)1β in synovium of patients with rheumatoid arthritis (RA) and its association with histological synovitis. Methods: This cross-sectional study recruited RA patients at the Department of Rheumatology, Sun Yat-Sen Memorial Hospital from May 2010 to October 2016. Clinical data were collected. Conventional radiographs of bilateral hands and wrists were performed and assessed according to Sharp/van der Heijde-modified Sharp score(mTSS). Synovial tissues from knee joints of all RA patients were obtained by biopsies, and then stained with HE and immunohistochemically for PGC-1β, CD3, CD20, CD38, CD68, CD15 and CD34 to evaluate synovitis, synovial PGC-1β expression and the densities of inflammatory cells and endothelial cells. The relationship between synovial PGC-1β expression and histological synovitis, disease activity and joint destruction in RA was analyzed by Spearman's rank correlation and multivariate linear regression. Results: There were 83 RA patients recruited with 20 (24.1%) males and 63 (75.9%) females, aged (54±14) years. PGC-1β expressed in the nuclei of lining synoviocytes, sublining inflammatory cells and vascular endothelial cells of RA synovium. The percentage of synovial PGC-1β+cells was positively correlated with histological synovitis score (r=0.333) and the densities of sublining CD3+ T cells (r=0.259), CD20+ B cells (r=0.320), CD38+plasma cells (r=0.342) and CD68+ macrophages (r=0.309)(all P<0.05). It was also positively correlated with erythrocyte sedimentation rate, C reactive protein and total mTSS (r=0.219-0.301, all P<0.05). Multiple linear regression analysis further confirmed the positive correlation between the percentage of synovial PGC-1β+cells and mTSS (β=0.312, P=0.004). Conclusion: Synovial PGC-1β is positively associated with local and systemic inflammation as well as joint destruction, which implies that PGC-1β might involve in the pathogenesis of synovitis and joint destruction in RA.

致残性全硬化性硬斑病（DPM）是一种罕见的严重致残性、儿童期多发的硬斑病，常累及四肢关节，造成真皮、皮下脂肪组织、筋膜、肌肉甚至骨骼硬化、炎症，导致不可逆性关节僵硬、恶病质、皮肤营养性溃疡合并感染等并发症。本病易误诊为SSc，但两者治疗策略和预后完全不同，在DPM炎症活动期甚至需要使用糖皮质激素冲击治疗。现报告1例成人起病患者的诊治经过，以期提高临床对该病的认识。

Objectives This study aims to investigate if addition of fibroblast-stromal cell markers to a classification of synovial pathotypes improves their predictive value on clinical outcomes in rheumatoid arthritis (RA). Methods Active RA patients with a knee needle synovial biopsy at baseline and finished 1-year follow-up were recruited from a real-world prospective cohort. Positive staining for CD20, CD38, CD3, CD68, CD31, and CD90 were scored semiquantitatively (0-4). The primary outcome was radiographic progression defined as a minimum increase of 0.5 units of the modified total Sharp score from baseline to 1 year. Results Among 150 recruited RA patients, 123 (82%) had qualified synovial tissue. Higher scores of CD20+ B cells, sublining CD68+ macrophages, CD31+ endothelial cells, and CD90+ fibroblasts were associated with less decrease in disease activity and greater increase in radiographic progression. A new fibroblast-based classification of synovial pathotypes giving more priority to myeloid and stromal cells classified samples as myeloid-stromal (57.7%, 71/123), lymphoid (31.7%, 39/123), and paucicellular pathotypes (10.6%, 13/123). RA patients with myeloid-stromal pathotype showed the highest rate of radiographic progression (43.7% vs. 23.1% vs. 7.7%, p = 0.011), together with the lowest rate of Boolean remission at 3, 6, and 12 months. Baseline synovial myeloid-stromal pathotype independently predicted radiographic progression at 1 year (adjusted OR: 3.199, 95% confidence interval (95% CI): 1.278, 8.010). Similar results were obtained in a subgroup analysis of treatment-naive RA. Conclusions This novel fibroblast-based myeloid-stromal pathotype could predict radiographic progression at 1 year in active RA patients which may contribute to the shift of therapeutic decision in RA.

目的:分析嗜酸性肉芽肿性多血管炎（EGPA）的临床特征，以提高对该病的认识。方法:收集2003年12月至2020年4月在中山大学孙逸仙纪念医院诊治的EGPA患者，诊断须符合1990年ACR分类标准，回顾性分析其人口学特征、临床表现、实验室和辅助检查等临床资料。采用 χ2检验和Mann-whitney U检验。 结果:52例EGPA患者男性34例（65.4%），发病中位年龄47（38~55）岁，发病到诊断中位时间30（4~96）个月。呼吸道（61.5%）和鼻/鼻窦表现（21.2%）首发多见，首诊以呼吸科（53.8%）为主，风湿科占11.5%，但44.2%患者经风湿科医生确诊。临床表现以哮喘（88.5%）、鼻/鼻窦（88.5%）、肺（76.9%）和神经系统受累（61.5%）多见。8例（15.4%）患者ANCA阳性，其哮喘比例低于ANCA阴性者，而全身症状尤其关节痛、肾脏受累比例、嗜酸粒细胞水平、ESR、CRP、伯明翰血管炎活动度评分、血管炎损伤指数均显著升高（ P<0.05）。有预后不良因素者占21.2%~34.6%。 结论:EGPA的早期诊断重要，ANCA阳性的患者病情可能更重，诊治应是以风湿科和呼吸科为基础的多学科合作。

Objective: To investigate the value of baseline anti-mutated citrullinated vimentin (MCV) antibody for predicting one-year radiographic progression in patients with rheumatoid arthritis (RA). Methods: Consecutive RA patients were recruited from November 2014 to July 2018 at Department of Rheumatology, Sun Yat-sen Memorial Hospital, Clinical data were collected including disease activity score in 28 joints with four variables including C-reactive protein (CRP).Serum anti-MCV antibody at baseline was detected by enzyme-linked immunosorbent assay. X ray assessment of both hands/wrists was performed and assessed according to the Sharp/van der Heijde modified score (mTSS) at baseline and the 12th month. Univariate and multivariate logistic regression analyses were used to identify the risk factors for one-year radiographic progression. Results: Among 220 RA patients recruited, the positive rate of anti-MCV antibody at baseline was 77.7%. Compared with those with negative anti-MCV antibody, RA patients with positive anti-MCV antibody had higher disease activity score in 28 joints with four variables induding CRP [3.8 (2.4, 5.0) vs. 3.1 (2.1, 4.0), P=0.007], more physical dysfunction (21.6% vs. 8.2%, P=0.033) and higher radiographic indicators including mTSS [11 (2, 27) vs. 4 (1, 10), P=0.003], joint space narrowing [JSN, 4 (0, 14) vs. 2 (0, 6), P=0.024] and joint erosion[JE, 5 (1, 18)vs. 3 (0, 5), P=0.003]. After one-year follow-up, sixty-six RA patients (30.0%) developed radiographic progression, the percentage of whom was significantly higher in positive anti-MCV group than that in negative anti-MCV group (33.9% vs.16.3%, P=0.018). Multivariate logistic regression analysis suggested that positive anti-MCV antibody at baseline was an independent risk factor for one-year radiographic progression (OR=2.341, 95%CI 1.002-5.469). Conclusion: Positive anti-MCV antibody at baseline predicts one-year radiographic progression in RA patients. In the future, anti-MCV antibody can be used not only as a supplementary laboratory marker, but also in disease activity assessment and prognosis prediction for RA.

本文报道一例罕见的确诊弥漫皮肤型系统性硬化症和高血压3年的女性患者，近2个月出现血尿、蛋白尿及血肌酐进行性升高，经肾活检病理确诊为合并显微镜下多血管炎引起的急进性肾小球肾炎，予糖皮质激素冲击及免疫抑制剂治疗后病情好转，未出现硬皮病肾危象。