Search

"Paper mills" are unethical outsourcing agencies proficient in fabricating fraudulent manuscripts submitted to scholarly journals. In earlier years, the activity of such companies involved plagiarism, but their processes have gained complexity, involving the fabrication of images and fake results. The objective of this study is to examine the main features of retracted paper mills' articles registered in the Retraction Watch database, from inception to the present, analyzing the number of articles per year, their number of citations, and their authorship network. Eligibility criteria for inclusion: retracted articles in any language due to paper mill activity. Retraction letters, notes, and notices, for exclusion. We collected the associated citations and the journals' impact factors of the retracted papers from Web of Science (Clarivate) and performed a data network analysis using VOSviewer software. This scoping review complies with PRISMA 2020 statement and main extensions. After a thorough analysis of the data, we identified 325 retracted articles due to suspected operations published in 31 journals (with a mean impact factor of 3.1). These retractions have produced 3708 citations. Nearly all retracted papers have come from China. Journal's impact factor lower than 7, life sciences journals, cancer, and molecular biology topics were common among retracted studies. The rapid increase of retractions is highly challenging. Paper mills damage scientific research integrity, exacerbating fraud, plagiarism, fake images, and simulated results. Rheumatologists should be fully aware of this growing phenomenon. [ABSTRACT FROM AUTHOR]

Background: Gout is the most common inflammatory arthritis with an increasing prevalence and incidence across the globe. We aimed to provide a comprehensive and systematic knowledge map of gout research to determine its current status and trends over the past decade. Methods: Publications on gout research were obtained from the Web of Science Core Collection (WOSCC) database. Bibliometric R, VOSviewer, and Citespace were employed to analyze the eligible literature. Results: A total of 5535 publications concerning gout research between 2012 and 2021 were included. Most publications and citations both numerically came from China. The strongest international cooperation belonged to the USA. The University of Auckland was the most productive institution with a leading place in research collaboration. The prime funding agency was the National Natural Science Foundation of China. Most papers were published in Clinical Rheumatology. Annals of the Rheumatic Diseases achieved the highest number of citations, H-index and IF, which showed the most excellent comprehensive strength. The individual author with the most paper authorship was Dalbeth Nicola with 241 publications and 46 H-index. Keywords and co-citation analysis discovered that pathological mechanism remains the future hotspot in gout research. It may involve gout connection with gut microbiota, NLRP3 inflammasome, xanthine oxidase, and urate-transporter ABCG2. In addition, besides metabolic diseases, the relationship between gout and heart failure may need more attention. Conclusion: This study clarified the current status and research frontier in gout over the past decade, which would provide valuable research references for later researchers. Key Points •We disclosed the current status and frontier directions of gout over the past 10 years worldwide. •We identified future hotspots of gout research, including gout connection with gut microbiota, NLRP3 inflammasome, xanthine oxidase, and urate-transporter ABCG2. •We discovered that the relationship between gout and heart status would be the research frontier. [ABSTRACT FROM AUTHOR]

Background: Bibliometric analysis is a mature method for quantitative evaluation of academic productivity. In view of the rapid development of research in the field of systemic lupus erythematosus (SLE) in the past decade, we used bibliometric methods to comprehensively analyze the literature in the field of SLE from 2013 to 2022. Methods: The relevant literature in the field of SLE from 2013 to 2022 was screened in the Web of Science Core Collection database. After obtaining and sorting out the data, CiteSpace and VOSviewer software were used to visualize the relevant data, and SPSS software was used for scientific statistics. Results: A total of 18,450 publications were included in this study. The number of articles published over the past 10 years has generally shown an upward trend, while Altmetric attention scores have also shown a clear upward trend in general and in most countries. Citation analysis and Altmetric analysis can mutually prove and supplement the influence of papers. The USA, China, Japan, Italy, and the UK are the most productive countries, but China and Japan are significantly inferior to other countries in terms of research influence. Four of the top ten authors are at the center of the collaboration network. LUPUS is the most contributing journal. The theme of systemic lupus erythematosus research mainly focuses on the pathogenesis, treatment, and management of SLE, and the emerging trend is related research on machine learning and immune cells. Conclusion: This study shows the research status of SLE, clarifies the main contributors in this field, discusses and analyzes the research hotspots and trends in this field, and provides reference for further research in this field to promote the development of SLE research. Key Points • Through bibliometric analysis, Altmetric analysis, and visual analysis, we reveal the global productivity characteristics of SLE-related papers in the past 10 years. • The number of global SLE-related studies has shown a significant increase, indicating that SLE is still a hot topic and deserves further study. • Citation analysis and Altmetric analysis can mutually prove and supplement the influence of papers, and the attention of related literature among non-professional researchers is increasing. • The theme of SLE research mainly focuses on the pathogenesis, treatment, and management of SLE. The emerging trend is machine learning and immune cells, which may provide new strategies for the diagnosis and treatment of SLE in the future. [ABSTRACT FROM AUTHOR]

This study aimed to report the most current data on the incidence and disability-adjusted life years (DALY) associated with osteoarthritis in China from 1990 to 2019. Publicly available modelled data from Global Burden of Disease Study (GBD) 2019 were used. The incidence and DALY, due to osteoarthritis in China, stratified by sex, trends of associated risk factors, assess the age, period, and cohort effects on the long-term trends of osteoarthritis incidence and DALY in China from 1990 to 2019. We found that the age-standardized incidence and DALY rates of osteoarthritis in China are higher than the average levels in Asia, Africa, and Oceania. In 2019, the number of cases of osteoarthritis in China was 10,681,311, an increase of 132.66% compared with 1990. the DALY of osteoarthritis in China was 4,724,885 person-years, which was 159.70% higher than that in 1990. In 2019, the incidence and DALY rates of osteoarthritis in China was 750.96/100,000,332.19/100,000. High body-mass as risk factors for osteoarthritis DALY with the population attributable proportion (PAF) increasing steadily from 1990 to 2019. The incidence and DALY rates of three types of osteoarthritis from high to low are osteoarthritis knee, osteoarthritis hand, and osteoarthritis hip. Age–period–cohort model showed that the incidence rate of osteoarthritis in China shows a trend of increasing first and then decreasing with age; concurrently, the DALY rate of osteoarthritis in China increased with age. For the period effect, we found that the period rate ratio (RR) of osteoarthritis incidence and DALY rates kept increasing in the cohort born before 2005–2009, and then, it was gradually reduced by year of birth in the cohort born after 2005–2009. As for cohort effect, the cohort RR of incidence rate of osteoarthritis almost has no change, while the cohort RR of DALY rate of osteoarthritis kept increasing from 1990 to 2019. The burden and impact of osteoarthritis in China are substantial and are increasing. Adopting suitable control and preventive community measures to reduce modifiable risk factors is needed to reduce the current and future burden of osteoarthritis in China. Key Points • This paper analyzes the disease burden of osteoarthritis in China for the first time and discusses the influence on the disease burden of osteoarthritis from the perspectives of age, period, and cohort. [ABSTRACT FROM AUTHOR]

Background: Melanoma differentiation-associated gene 5 (MDA5), as a cytoplasmic sensor for viral double-stranded RNAs, has received increasing attention in recent years. Although considerable headway has been made on the functional role of MDA5 in antiviral immunity and autoimmune disease, the available literature is insufficient to assess the vast field. Methods: This study performed a bibliometric analysis to investigate current hotspots in the global scientific output of MDA5 over the past two decades. Related publications and recorded information from 2002 to 2022 in the Web of Science Core Collection (WoSCC) database were retrieved. VOSviewer and CiteSpace were used for quantitative evaluation and visualization. Results: A total of 2267 original articles and reviews were obtained, and the annual number of publications related to MDA5 was increasing rapidly. China has published the most papers, while the USA was the most influential country with the most citations and the highest H-index. The Chinese Academy of Sciences, the United States Department of Health and Human Services, and the Journal of Virology were the most prolific research affiliation, funding source, and journal, respectively. Fujita T (Kyoto University) was the most productive author with the highest H-index and had close cooperation with Kato H and Yoneyama M. The keywords "RIG-I," "MDA5," "innate immunity," "double-stranded-RNA," and "recognition" had the highest frequency, while "dermatomyositis" as well as "autoantibody" seemed to be the emerging hotspots. Conclusion: This study comprehensively demonstrated the research frontiers of MDA5 and will provide a useful resource for scholars to conduct future decisions. Key Points: We conducted the first in-depth survey of the research frontiers on melanoma differentiation-associated gene 5 (MDA5) over the past two decades via bibliometric analysis. We found that many early breakthroughs have been made in the mechanism of MDA5-mediated antiviral immune responses, and the role of MDA5 in autoimmune and autoinflammatory diseases has raised the recent concern. We identified that the virus infection-associated pathogenesis and effective therapeutic strategy of anti-MDA5 antibody-positive dermatomyositis will remain the hotspots in the future. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) pneumonia, firstly reported in Wuhan, Hubei province, China, has rapidly spread around the world with high mortality rate among critically ill patients. The use of corticosteroids in COVID-19 remains a major controversy. Available evidences are inconclusive. According to WHO guidance, corticosteroids are not recommended to be used unless for another reason. Chinese Thoracic Society (CTS) proposes an expert consensus statement that suggests taking a prudent attitude of corticosteroid usage. In our clinical practice, we do not use corticosteroids routinely; only low-to-moderate doses of corticosteroids were given to several severely ill patients prudently. In this paper, we will present two confirmed severe COVID-19 cases admitted to isolation wards in Optical Valley Campus of Tongji hospital, Tongji Medical College, Huazhong University of Science and Technology. We will discuss questions related to corticosteroids usages. [ABSTRACT FROM AUTHOR]

With the financial support from International League of Associations for Rheumatology (ILAR), we held a rheumatologic care education project in Xishuang Ban'na, a remote district in southern China. During 2014-2015, our project devoted to improve rheumatologic care skills of local clinical practitioners and basic rheumatologic knowledge tothe public. This paper summarizes the undeveloped conditions of rheumatology and our effort in Xishuang Ban'na. [ABSTRACT FROM AUTHOR]

This review introduces the history of development, organizational structure, funding resources, data collection, and quality control of the Chinese Rheumatism Data Center (CRDC) and summarizes the collection of data. In 2009, Peking Union Medical College Hospital (PUMCH), together with several rheumatism centers, established the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research Group (CSTAR) to collect data on Chinese patients for the study of SLE disease characteristics. In 2011, CSTAR was extended with the formation of the CRDC at PUMCH with direction from the National Health and Family Planning Commission of the PRC. The CRDC currently includes 300 registration sites and 50 regional sites that have successively begun to collect data on 12 rheumatic diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, dermatomyositis, Takayasu arteritis, IgG4-related diseases, ANCA-associated vasculitis, gout, polyarteritis nodosa, unclassified systemic vasculitis, and Behcet disease. To date, 17,224 patients have been enrolled in the CRDC. Based on the SLE patients registered in the CRDC, papers investigating basic demographic characteristics and first symptom in Chinese SLE patients, risk factors of pulmonary hypertension, correlations between autoantibodies and clinical manifestations, and factors related to fetal loss have been published. The CRDC is a national registry that provides real-life data to improve clinical decision-making. At the same time, without additional work for the clinician, the CRDC is a powerful research database. The CRDC database provides sufficient information for Chinese clinical studies on rheumatology. Moreover, a mobile device application ensures convenient and efficient data collection without compromising data quality, thereby providing strong evidence-based data for the diagnosis and treatment of Chinese rheumatic patients. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to compare the differences among patients of different onset ages in various subtypes of lupus erythematosus (LE) and to draw a panorama of the clinical characteristics of patients with different onset ages. Method: Subjects were recruited from the Lupus Erythematosus Multicenter Case–control Study in Chinese populations (LEMCSC), grouped by the age of onset (childhood-onset: onset < 18 years, adult-onset: onset 18–50 years, late-onset: onset > 50 years). The data collected included demographic characteristics, LE-related systemic involvement, LE-related mucocutaneous manifestations, and laboratory results. All included patients were assigned into three groups: systemic LE (SLE) group (with systemic involvement, with or without mucocutaneous lesions), cutaneous LE (CLE) group (patients who were accompanied by any type of LE-specific cutaneous manifestations), and isolated cutaneous LE (iCLE) group (patients who were in CLE group without systemic involvements). Data were analyzed using R version 4.0.3. Results: A total of 2097 patients were involved, including 1865 with SLE and 232 with iCLE. We also identified 1648 patients with CLE among them, as there was some overlap between the SLE population and CLE population (patients with SLE and LE-specific cutaneous manifestations). Later-onset lupus patients seemed to be less female predominance (p < 0.001) and have less systemic involvement (except arthritis), lower positive rates of autoimmune antibodies, less ACLE, and more DLE. Moreover, childhood-onset SLE patients presented a higher risk of LE family history (p = 0.002, vs adult-onset SLE). In contrast to other LE-nonspecific manifestations, the self-reported photosensitivity history decreased with the age of onset in SLE patients (51.8%, 43.4%, and 39.1%, respectively) but increased in iCLE patients (42.4%, 64.9%, and 89.2%, respectively). There was also a gradual increase in self-reported photosensitivity from SLE, CLE, to iCLE in both adult-onset and late-onset lupus patients. Conclusions: A negative correlation was suggested between the age of onset and the likelihood of systemic involvement, except for arthritis. As the age of onset increases, patients have a greater propensity to exhibit DLE compared to ACLE. Moreover, the presence of rapid response photodermatitis (i.e., self-reported photosensitivity) was associated with a lower rate of systemic involvement. Trial registration: This study was registered with the Chinese Clinical Trial Registry (registration number: ChiCTR2100048939) on July 19, 2021, retrospectively registered. Key Points • We confirmed some phenomena that have been found in patients with SLE, such as the highest proportion of females of reproductive age, the higher risk of LE family history in childhood-onset SLE patients, and the less self-reported photosensitivity in the late-onset SLE group. We also compared the similarities and differences of these phenomena in patients with CLE or iCLE for the first time. • In patients with SLE, the proportion of females peaked in adult-onset patients, but this phenomenon disappeared in iCLE patients: the female-male ratio tends to decrease from childhood-onset iCLE, adult-onset iCLE, to late-onset iCLE. • Patients with early-onset lupus are more likely to have acute cutaneous lupus erythematosus (ACLE), and patients with late-onset lupus are more likely to have discoid lupus erythematosus (DLE). • In contrast to other LE-nonspecific manifestations, the incidence of rapid response photodermatitis (i.e., self-reported photosensitivity) decreased with the age of onset in SLE patients but increased with the age of onset in iCLE patients. [ABSTRACT FROM AUTHOR]

Objective: To summarize the clinical, serological, and radiological characteristics of anti-synthetase syndrome (ASS) patients with different anti-aminoacyl-tRNA synthase antibody. Methods: Retrospective analysis was performed based on the clinical data of 88 patients diagnosed with ASS in Tianjin Medical University General Hospital from January 2015 to December 2020. The clinical data included general conditions, serological indexes, high-resolution CT (HRCT) characteristics, and pulmonary function manifestations. Results: Among the 88 patients, there were 17 males and 71 females. The anti-synthetase antibodies included anti-Jo-1 (n = 42), anti-PL-7 (n = 14), anti-PL-12 (n = 9), anti-EJ (n = 20), and anti-OJ (n = 3) antibodies. The most common clinical manifestations of ASS patients were interstitial lung disease (ILD) (90%, 79/88), followed by myositis (79.5%, 70/88), arthritis (50%, 44/88), and rash (50%, 44/88). The frequency of arthritis in the anti-Jo-1 antibody–positive group was higher than that of the anti-PL-7 and anti-EJ antibody groups (P = 0.004, P = 0.002, respectively). The frequency of Gottron's sign in the anti-PL-7 antibody positive group was higher than that of the anti-Jo-1 and anti-PL-12 antibody–positive groups (P = 0.006, P = 0.04). Isolated arthritis was the most frequent initial symptoms in anti-Jo-1 antibody–positive group (47.6%, 20/42), while isolated ILD was most frequent in patients with anti-EJ antibody (50%, 10/20), and isolated myositis in patients carrying anti-OJ (66.7%, 2/3). There were only 32 cases (36.4%) with the typical clinical triad (myositis, arthritis, ILD). In our cohort, 79 patients (90%) were complicated with ILD. Meanwhile, 7 out of 79 cases were classified into rapid progressive group with 6 cases (85.7%) carrying anti-Ro-52 antibody. The probability of reticular and honeycombing shadow in HRCT of patients with anti-EJ antibody positive was higher than that of other groups (P < 0.05). Conclusion: ILD, myositis, and arthritis were the most common clinical manifestations in patients with ASS. Different antibody-positive patients have different initial symptoms. Patients with isolated arthritis, myositis, and ILD should be vigilant of ASS. The complication of anti-Ro-52 antibody in ASS patients was associated with rapidly progressive pulmonary interstitial disease. Patients with positive anti-EJ antibodies tend to have ILD as the first symptom, and with high occurrence of ILD, the HRCT showed more serious patterns, suggesting the correlation between anti-EJ antibodies and ILD. Key Points • Analyzing specific clinical manifestations in ASS patients with different ARS antibodies can raise awareness of the disease and reduce misdiagnosis. • Anti-EJ antibodies were correlated with ILD. • Anti-Ro-52 antibodies may correlate with RP-ILD in ASS patients. [ABSTRACT FROM AUTHOR]

Several studies have demonstrated the benefits of thalidomide as a treatment for patients with ankylosing spondylitis (AS); however, published literature reported controversial results. We conducted a meta-analysis to systematically evaluate the efficacy of thalidomide in AS patients. PubMed, Embase, Cochrane Library, Web of Science, Wanfang Data, and China National Knowledge Infrastructure (CNKI) were searched for relevant studies. The Q test and I2 statistic were used to examine between-study heterogeneity. Fixed- or random-effects models were selected based on study heterogeneity. The risk difference (RD), absolute risk reduction (ARR), and weighted mean difference (WMD) with 95% confidence intervals (CI) were pooled for dichotomous or continuous data, as appropriate. Sensitivity analyses, funnel plots, and the Begg's tests were also performed. Overall, 19 trials with 1471 patients were included. The effectiveness of thalidomide alone and combined with other drugs was significantly higher than the control group, and the pooled RDs were 0.15 (95% CI: 0.10–0.20, I2 = 0%) and 0.20 (95% CI: 0.14–0.25, I2 = 13.4%), respectively. Thalidomide treatment yielded significant improvements in secondary outcomes for patients with AS. The adverse reaction rate for thalidomide alone was low than that for the control group (ARR = 0.08, 95% CI: 0.01–0.15, I2 = 0.0%), while there was no significant difference in the safety between the group in which thalidomide was combined with other drugs and the control (ARR = 0.03, 95% CI: − 0.04–0.10, I2 = 41.1%). The findings suggest that thalidomide improves the effectiveness of AS treatment, which should be considered by physicians. However, owing to the inclusion of several low-quality and Chinese studies, additional rigorous randomized controlled trials (RCTs) are needed in the future to confirm the results of this meta‑analysis. [ABSTRACT FROM AUTHOR]

Objectives: CXCL13 levels have been reported to be elevated in primary Sjögren's syndrome (pSS) patients. This study investigated alterations involving the CXCL13/CXCR5 axis in pSS patients and explored the correlation between CXCL13 expression and disease severity. Methods: Six databases, including PubMed, Web of Science, Embase, Ovid Medline, China National Knowledge Infrastructure, and Wanfang, were searched. The quality of the included studies was assessed using the Newcastle-Ottawa Scale criteria. We analyzed CXCL13 concentrations in the serum and saliva of pSS patients and healthy controls (HCs). Next, we analyzed the percentage of CXCR5+CD4+ T cells among CD4+ T cells in pSS patients and HCs. Correlations between CXCL13 levels and serological, clinical, and histological parameters of patients were also assessed. Publication bias was determined using funnel plots and Egger's test. Results: Twenty-three articles were included; of these, 14 studies reported CXCL13 levels, and 5 reported CXCR5+CD4+ T cell count of pSS patients and HCs. Nine articles covering 32 studies reported correlations between serum CXCL13 levels and serological, clinical, and histological parameters in pSS patients. Expressions of CXCL13 and CXCR5+CD4+ T cell count were significantly increased in pSS patients compared with those in HCs. There was a positive correlation between CXCL13 levels and serum IgG levels, disease activity indices, and focus score in minor salivary gland biopsy of patients. Conclusions: CXCL13 abundance was evidently elevated in pSS patients and correlated with disease activity and can therefore probably be employed as a robust biomarker to monitor and diagnose pSS. Key Points • Serum CXCL13 is significantly elevated in primary Sjögren's syndrome patients. • CXCL13 level correlates with disease severity and activity of primary Sjögren's syndrome. • CXCL13 can probably be used as a robust biomarker to monitor and diagnose primary Sjögren's syndrome. [ABSTRACT FROM AUTHOR]

Objectives: To explore the relationship among patient-specific SNPs from one SLE family, lupus susceptibility, and laboratory indicators in a western Chinese population. Methods: We previously performed whole exome sequencing in one SLE family and screened 5 SLE candidate SNPs. In this study, we verified them in 634 SLE patients and 400 healthy controls and analyzed the relationship between SNPs and laboratory indicators. Results: Among the 5 candidate SNPs, PHLDB1 rs7389T/G (dominant model, OR = 0.627, 95%CI = 0.480–0.820, P = 0.001) and WDFY4 rs7097397G/A (dominant model, OR = 0.653, 95%CI = 0.438–0.973, P = 0.035) were associated with SLE susceptibility. In addition, the G allele of rs7389 was related to an increased level of TNF-α (q = 0.013). The A allele of rs7097397 was related to reduced levels of IL-1β (q = 0.033) and IL-6 (q = 0.039) and high positive rate of antinuclear antibodies (q = 0.021). Conclusions: Our study indicated that both the rs7389T/G and rs7097397G/A polymorphisms were related to SLE susceptibility in western China. rs7389T/G was related to increased TNF-α content, while rs7097397G/A was associated with reduced IL-1β and IL-6 content and increased antinuclear antibody positive rate. Key Points • The G allele of rs7389 was related to reduced susceptibility to SLE. • The A allele of rs7097397 was associated with reduced susceptibility to SLE. • The G allele of rs7389 was related to increased levels of TNF-α. • The A allele of rs7097397 was related to decreased concentrations of IL-1β and IL-6, as well as an increased positive rate of antinuclear antibody. [ABSTRACT FROM AUTHOR]

Objective: Left ventricular diastolic dysfunction (LVDD) is a common manifestation of cardiac involvement in systemic sclerosis (SSc), which is associated with increased mortality, but little is known about the risk factors. The aim is to determine the frequency and potential predictors of SSc-LVDD. Methods: We conducted a prospective multi-center cohort study, enrolling 784 SSc patients assessed by echocardiography between April 2008 and June 2019. Diagnosis of systemic sclerosis was according to the 2013 American College of Rheumatology (ACR)/the European League Against Rheumatism (EULAR) classification criteria. Data were compared between patients with and without LVDD, while univariate and multivariate regression analysis was performed to determine the factors independently associated with LVDD. Results: LV diastolic dysfunction was present in 246/784 (31.4%) of the subjects. There were no significant differences in gender, BMI, or disease duration between the two groups. Around 40% of the patients in the SSc-LVDD group and in the SSc-non LVDD group had diffused cutaneous involvements. Factors independently associated with LV diastolic dysfunction in multivariable analysis included age at onset (OR 1.053, 95%CI 1.021–1.086, p = 0.001), pulmonary arterial hypertension (OR 3.057, 95%CI 1.468–6.367, p = 0.003), positivity of anti-RNP antibody (OR 2.455, 95%CI 1.049–5.745, p = 0.038), increased WBC count (OR 1.156, 95%CI 1.037–1.287, p = 0.009), elevated levels of uric acid (OR 1.003, 95%CI 1.000–1.006, p = 0.036), and triglyceride (OR 1.515, 95%CI 1.106–2.077, p = 0.010). Conclusion: LV diastolic dysfunction was prevalent in the SSc population. Advanced onset age, PAH, positive anti-RNP antibody, increased WBC count, and adverse metabolic status were independent risk factors for SSc-related LVDD. Key Points • In this Chinese multi-center cohort of systemic sclerosis, LVDD is not a rare complication, with a prevalence of 31.4%. • The presence of advanced onset age, PAH, positive anti-RNP antibody, increased WBC count and adverse metabolic status were baseline predictors of developing LVDD in SSc. [ABSTRACT FROM AUTHOR]

Objectives: Takayasu arteritis (TA) is a rare granulomatous vasculitis. Numano type V was the most frequently involved arteritis in China. In our study, we aimed to investigate the characteristics of the type V TA in Chinese people. Methods: We retrospectively reviewed 143 medical charts of TA patients who were admitted to Beijing Anzhen Hospital from 2012 to 2019. All statistical studies were carried out with the SPSS program V.24.0. Results: The duration of disease at first admission of type V TA patients was longer than non-type V group (110.6 (21.0, 168.0) vs. 69.1 (12.0, 120.0) months, P = 0.034). The incidences of hypertension and coronary artery involvement were more common in type V TA patients compared with non-type V patients (59.5% vs. 30.4%, P < 0.001; 33.8% vs. 17.4%, P = 0.025; respectively). The numbers of platelet (PLT) and the plateletcrit (PCT) in type V TA patients were significantly smaller than those in non-type V group (244.9 (190.5, 293.8) vs. 281.6 (214.5, 332.0) × 109/L, P = 0.005; 0.25 (0.20, 0.28) vs. 0.28 (0.22, 0.32) %, P = 0.003; respectively). Lesions located in the left anterior descending coronary (LAD) and the left circumflex coronary (LCX) presented more frequently in type V TA patients than in non-type V patients (LAD 76.0 vs. 41.7%, P = 0.041; LCX 56.0 vs. 8.3%, P = 0.006; respectively). Conclusions: Type V TA patients had longer disease duration and higher incidence of hypertension and were more prone to have coronary artery involvement compared with non-type V patients. Key Points • This study is the first to assess characteristics of the type V TA in Chinese people. • Type V TA patients had longer disease duration and higher incidence of hypertension and were more prone to have coronary artery involvement compared with non-type V patients. • The numbers of platelet (PLT) and the plateletcrit (PCT) in type V TA patients were significantly smaller than those in non-type V group. [ABSTRACT FROM AUTHOR]

Objectives: Fatigue is a common symptom in patients with ankylosing spondylitis (AS). However, fatigue of AS patients has not been well elucidated in China. This study aimed to evaluate the predictors of fatigue and the effects of fatigue on health-related quality of life among patients with AS. Method: A total of 150 AS patients were involved in the study. A series of questionnaires included the following: Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Metrology Index (BASMI), Bath Ankylosing Spondylitis Functional Index (BASFI), the 10-cm Visual Analog Scale (VAS), the Self-Rating Anxiety Scale (SAS), the Self-Rating Depression Scale (SDS), the Pittsburgh Sleep Quality Index (PSQI), the Health Assessment Questionnaire-Disability Index (HAQ-DI), the Short Form 36 Health Survey (SF-36), and the Fatigue Severity Scale (FSS). Independent samples t test, Mann-Whitney U test, chi-square analysis, Pearson/Spearman correlation, and binary logistic regression were used to analyze the data. Results: The results demonstrated that 48.7% individuals with AS suffered from fatigue. Binary logistic regression indicated that waist-to-hip ratio, BASDAI, and sleep disturbance were independent predictors of fatigue in AS patients. Meanwhile, severe fatigue led to lower quality of life. Conclusion: These findings suggested that medical personnel should pay more attention to AS patients with fatigue and take effective measures to relieve fatigue. Key Points • Incidence of fatigue in AS patients is 48.7% according to this cross-sectional study. • The occurrence of fatigue was associated with higher WHR, higher BASDAI, and sleep disturbance. • We also found that the occurrence of fatigue significantly reduced the quality of life in AS patients both physically and psychologically. [ABSTRACT FROM AUTHOR]

Objective: The main purpose of this study was to investigate the current situation of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) in China. The relationships between social demography, disease activity, psychological status, clinical variables, and ILD were analyzed. Besides that, the quality of life (QoL) in pSS with ILD was also analyzed. Methods: In the cross-sectional study, 101 pSS patients participated in this study. Under the guidance of the researchers, the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren's Syndrome Patient-Reported Index (ESSPRI), the Short-Form 36 health survey (SF-36), Hospital Anxiety and Depression Scale (HADS), Pittsburgh sleep quality index (PSQI), Summated Xerostomia Inventory (SXI), and other related questionnaires were completed. Independent sample t tests, Mann-Whitney U test, chi-square test, and correlational analysis were used. Results: The data showed that 28 (30.1%) pSS people with ILD. The occurrence and development of ILD were associated with disease duration, fatigue, alanine aminotransferase (ALT), neutrophils, albumin, and use of hormone. The survey also found that the QoL of pSS-ILD was significantly lower than non-ILD, especially the score in the dimension of role physical function. Conclusions: ILD was very common in pSS. ILD had a significant negative impact on the QoL of patients. Therefore, it is of great significance to strengthen the early intervention and drug treatment of pSS patients to prevent ILD and improve their QoL. Key Points • This cross-sectional study finds that the incidence of ILD in patients with pSS is 30.1%. • The occurrence and development of ILD is associated with disease duration, fatigue, alanine aminotransferase (ALT), neutrophils, albumin, and use of hormone. •The study also finds that the QoL of pSS-ILD is significantly lower than in patients with non-ILD, especially the score in the dimension of role physical function. [ABSTRACT FROM AUTHOR]

Objective: Due to lack of comprehensive evaluation for various detection methods for antineutrophil cytoplasmic antibody (ANCA) in Chinese population, we evaluate the diagnostic performance of 12 established analysis methods in Chinese patients having granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Methods: Sera were collected from 209 patients with GPA or MPA and 243 diseases controls from 15 centers. Twelve different reagents were employed for C-ANCA, P-ANCA, myeloperoxidase (MPO)-ANCA, and proteinase 3(PR3)-ANCA detection. The accuracy, sensitivity, and specificity of each method were analyzed. Results: The accuracy of the two indirect immunofluorescence (IIF) and two line immunoassay (LIA) was 0.838 and 0.874, 0.869, and 0.862, respectively. The accuracy of the eight quantitative antigen-specific immunoassays was varied from 0.867 to 0.967. The sensitivity of ANCA-associated vasculitis (AAV) was 0.770 and 0.761 for the two IIF, 0.727 and 0.718 for the two LIAs, respectively. For the eight quantitative antigen-specific immunoassays, the sensitivity varied from 0.79 to 0.967. The specificity was 0.897 and 0.971 for the two IIF, 0.992 and 0.988 for the two LIAs, respectively. For the eight quantitative antigen-specific immunoassays, the specificity of AAV varied from 0.963 to 0.983. Conclusion: For Chinese patients suspected of having GPA and MPA, both the first-generation enzyme-linked immunosorbent assay (ELISA) and high-quality antigen-specific immunoassay can be used to detect MPO-ANCA and PR3-ANCA alone, without the combined detection with IIF to have good diagnostic performance. The chemiluminescent immunoassay (CLIA) seems to be a method worth recommending. Key points • Quantitative antigen-specific immunoassays can be used to detect MPO-ANCA and PR3-ANCA without IIF in Chinese. • CLIA has the maximum AUC value and the minimum LR (−) value, which seems to be a method worth recommending. [ABSTRACT FROM AUTHOR]

Objectives: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by obstetric complications and thrombotic events associated with antiphospholipid antibodies (aPL). We aimed to compare the clinical characteristics and treatment of primary APS (PAPS) and secondary APS (systemic lupus erythematosus-APS, SAPS) patients and investigate risk factors associated with obstetric complications in Shanghai, China. Methods: We retrospectively collected and analyzed the data of obstetric APS (OAPS) patients from 2000 to 2017 in the APS-Shanghai (APS-SH) database. Results: One hundred eighty OAPS patients with a total of 450 pregnancies were included in this study. Two hundred twenty-one (49.11%) pregnancies resulted in miscarriage, and 161 (35.77%) pregnancies resulted in intrauterine death. In our cohort, when women were treated, 57 out of 66 pregnancies resulted in live births (86%). Of the 9 treated patients who failed to have live births, 3 had intrauterine deaths, 3 had fetal growth restriction, 2 had pneumorrhagia of the newborn, and 1 had a miscarriage. OAPS patients were divided into two groups: PAPS and SAPS. More SAPS patients than PAPS patients used glucocorticoids (GCs) and hydroxychloroquine (both p < 0.001). However, there was no significant difference in the GC dosage between SAPS and PAPS patients (p = 0.188). Lupus anticoagulant (LAC) and IgG aβ2GPI were risk factors for miscarriage (odds ratio (OR) = 2.398, 95% confidence interval (CI) = 1.276–4.505, p = 0.002; OR = 2.907, 95% CI = 1.558–5.405, p = 0.001, respectively) and intrauterine death (OR = 2.439, 95% CI = 1.299–4.580, p = 0.006; OR = 2.060, 95% CI = 1.089–3.897, p = 0.026, respectively). Conclusions: The live birth rate of OAPS patients in Shanghai was 86%. Even if OAPS patients were treated, pregnancy complications could occur, and these patients might need further second-line treatment. Key Points • This is the first study to report data on Chinese OAPS patients. The live birth rate was 86%. • Lupus anticoagulant and IgG aβ2GPI were risk factors for miscarriage and intrauterine death in our cohort. • Despite active treatment, 9 patients had obstetric complications. Therefore, further second-line treatment is still needed. [ABSTRACT FROM AUTHOR]

We aimed to systematically evaluate the accuracy of nCD64 in diagnosing infection in patients with autoimmune diseases. Studies were searched in PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, Wanfang, and Chongqing VIP databases up to October 2018. There was no restriction for language and age. Prospective studies examining the accuracy of nCD64 in diagnosing infection in patients with autoimmune diseases were included. The Quality Assessment of Diagnostic Accuracy Studies-2 tool was used to assess the quality of eligible studies. Stata 15.1 and Meta-DiSc 1.4 software were used for data analysis. Eleven studies fulfilled the inclusion criteria (677 patients, 229 patients with bacterial infection, and 448 without infection). The pooled sensitivity and specificity of nCD64 were 89% (95% confidence interval (CI) 82–93) and 94% (95% CI 91–96), respectively. The pooled positive likelihood ratio and negative likelihood ratio were 14.9 (95% CI 9.3–23.8) and 0.12 (95% CI 0.07–0.20), respectively. The diagnostic odds ratio and area under the summary receiver operating characteristic curve were 123 (95% CI 53–283) and 0.97 (95% CI 0.95–0.98), respectively. The univariate meta-regression analysis showed that region, type of disease, antibiotic therapy, and presentation of nCD64 measurement results were responsible for the heterogeneity. The Deeks' funnel plot asymmetry test showed that there was no publication bias (p = 0.15). nCD64 has a good overall diagnostic performance for differentiating infection from disease flare in patients with autoimmune diseases. Further studies are needed to confirm the optimized cutoff value. [ABSTRACT FROM AUTHOR]

Chronic exposure to high altitude may lead to hyperuricemia. We investigated the prevalence of hyperuricemia and its risk factors among employees in high-altitude areas. A cross-sectional survey with cluster sampling was performed at 23 worksites on the Qinghai-Tibet Plateau. Subjects were evaluated by using questionnaires, anthropometric and laboratory measurements, and abdominal ultrasound. A multilevel logistic regression model and restricted cubic spline were used to explore the associated factors of hyperuricemia. Of the 4198 employees included in the study, the age-standardized prevalence of hyperuricemia was 28.1% (95% confidence interval (CI) 26.7–29.5), with 31.9% (95%CI 30.2–33.6) in men and 17.9% (95%CI 15.7–20.1) in women. Hypertension, hyperlipidemia, and a meat-food pattern were positively associated with hyperuricemia in both sexes, while diabetes, shift work, body mass index, non-alcoholic fatty liver disease, and low intake of fruits and vegetables were significantly associated with hyperuricemia only in men. Furthermore, a nonlinear dose-response association between the number of cardiovascular risk factors (CRFs) and hyperuricemia was observed. Compared with those having 0 CRFs, the full-adjusted odds ratios (ORs) and 95%CIs for 1, 2, and ≥ 3 CRFs were 1.76 (95%CI 1.25–2.47), 2.54 (95%CI 1.81–3.55), and 3.05 (95%CI 2.16–4.31) in men, respectively, and 2.13 (95%CI 1.43–3.17), 2.78 (95%CI 1.71–4.53), and 3.13 (95%CI 1.50–6.55) in women, respectively. Hyperuricemia is common in employees at high-altitude areas. However, working at higher altitudes does not mean necessarily higher risk of hyperuricemia, as dietary factors and clustered CRFs are more significant. Thus, workplace-based lifestyle modifications should be promoted. [ABSTRACT FROM AUTHOR]

Objectives: The standard strategy for treating lupus nephritis comprises glucocorticoids together with either intravenous cyclophosphamide or oral mycophenolate mofetil, but the low remission rate is still a challenge in practice. This study was aimed to seek higher remission rate of lupus nephritis using a combined strategy.Method: A 24-week trial was conducted in 17 rheumatology or nephrology centers in China. A total of 191 lupus nephritis patients were randomized to follow a combined immunosuppressive treatment (CIST) with intravenous cyclophosphamide, an oral immunosuppressive agent, namely mycophenolate mofetil, azathioprine or leflunomide, and hydroxychloroquine (n = 95), or receive intravenous cyclophosphamide alone (n = 96) for 24 weeks. Glucocorticoid was given to both groups. The primary end point was a complete remission with a most stringent standard as proteinuria < 150 mg per 24 h, normal urinary sediment, serum albumin, and renal function at 24 weeks. The secondary end point was treatment failure at 24 weeks.Results: At week 24, both the rate of complete remission (39.5%) and total response (87.2%) was higher in the combined group, compared with CYC group (20.8% and 68.8%, p < 0.05). The cumulative probability of complete remission was also higher in the combined group (p = 0.013). In addition, the combined treatment was superior to routine CYC with less treatment failure (12.8% vs.31.2%, p < 0.001). No difference was found between the incidences of severe adverse events in the two arms: 3.2% (3/95 combined group) vs.4.2% (4/96 CYC group).Conclusion: Treatment with a combined immunosuppressive agent is superior to routine CYC only therapy in lupus nephritis. [ABSTRACT FROM AUTHOR]

The study aimed to determine whether unique clinical patterns of AS may exist in China, specifically to explore the different clinical manifestations caused by gender, HLA-B27 status, and age at disease onset. The multicenter cross-sectional survey was conducted and 1251 patients were enrolled across China, representing a broad spectrum of Chinese AS patients. The mean age at onset and diagnosis were 29.2 (11.4) and 33.5 (12.6) years, respectively. The male/female ratio was 2.7:1. Acute anterior uveitis (AAU) was experienced in 10.3% of AS patients and 9.1% patients had juvenile-onset AS (JoAS). Men were significantly younger at onset and diagnosis and showed a higher frequency of HLA-B27 positivity, JoAS, and AAU than women. HLA-B27-positive patients had a younger age of onset than HLA-B27-negative patients. HLA-B27-positive patients were nearly three times as likely to develop AAU than negative patients ( P = 0.04). JoAS patients had a family history of AS more often than adult-onset AS (AoAS) patients, and 4.9% of JoAS patients underwent surgical treatments, a rate more than six times that of AoAS patients ( P = 0.01). Men had higher levels of C-reactive protein than women, as did HLA-B27 positives compared to negative patients, and JoAS compared to AoAS (all P < 0.05). The clinical patterns of our AS patients were similar to those in other studies in non-Chinese cohort: (1) the age at onset was 29.2 (11.4) years, which was older than found in other studies; (2) men were more likely be HLA-B27 carriers than women; and (3) AAU was less common in Chinese patients. [ABSTRACT FROM AUTHOR]

This study aims to investigate the disease-related knowledge of gout patients and doctors in south China and to identify the important targets of education for patients and doctors. A cross-section survey of 154 primary gout patients and 185 doctors who may see gout patients was conducted with a modified questionnaire with ten items of gout-related knowledge. The participants were considered to have gout-related knowledge if he or she correctly answered seven or more items. One hundred and forty-nine valid questionnaires from patients, 33 from rheumatology physicians, and 151 from non-rheumatology doctors were collected for statistical analysis. The mean correctly answered items of three groups were 6.6 ± 2.2, 9.6 ± 0.53, and 8.0 ± 1.4, with rate of being considered to have knowledge about gout 51.7, 100, and 90.1 %, respectively ( P < 0.05). The correct answer rate for each particular item was over 80 % in the rheumatology physician group. Patients or non-rheumatology doctors knew the optimal serum uric acid (sUA) level (48.3 vs 55.6 %), the need to take lifelong urate-lowering drugs (29.5 vs 43.6 %), that allopurinol is a urate-lowering drug (55.7 vs 76.0 %), and how to prevent attacks induced by urate-lowering therapy (ULT) (60.4 vs 74.0 %). Logistic regression showed that higher education predicted which patients had gout-related knowledge. Both the gout patients and non-rheumatology doctors in south China had poor knowledge on ULT. Since many gout patients do not see rheumatologists, our data suggest that further education should focus on patients and non-rheumatologists and emphasize the use of urate-lowering drugs, treatment duration, the target sUA level, and prophylaxis against acute attacks. [ABSTRACT FROM AUTHOR]

The aim of this study is to explore the survival rate and risk factors of mortality in patients with late-onset systemic lupus erythematosus (SLE) in a large cohort. Clinical presentations, disease activity, organ damage scores, autoantibody profile, and mortality data were obtained retrospectively from late-onset SLE patients (onset age ≥50 years) diagnosed between 1995 and 2009. The risk factors of organ damage were evaluated by the chi-square test and logistic regression. The cumulative rate of survival was calculated by Kaplan-Meier method, and factors predictive of mortality were studied by Cox proportion hazard regression model. A total of 158 patients (132 female and 26 male) were studied. The average onset age was 58.66 ± 6.38 years and mean disease duration was 63.85 ± 48.17 months. One hundred and four patients had organ damage at the time of data analysis. Hematological system and kidney involvement were most common. Central nervous system involvement was relatively rare. In univariate logistic analysis, associations were found between SLE disease activity index (SLEDAI) at diagnosis (OR = 1.133, P = 0.001); renal involvement (OR = 2.441, P = 0.009) and edema (OR = 2.812, P = 0.003) were associated with organ damage. And SLEDAI at diagnosis (OR = 1.103, P = 0.034) was independent factor for organ damage in multivariate logistic regression. During the follow-up, 64 patients (51 female and 13 male) died. Five-, 10-, and 15-year survival rates were 80.4, 56.5, and 31.7 %, respectively. Median survival time was 123 months. The analysis of Cox proportion hazard regression model showed that age at disease onset (OR = 1.069, P = 0.002), compliance of medical care (OR = 3.282, P = 0.001), and SLEDAI at diagnosis (OR = 1.091, P = 0.003) were independent risk factors of mortality. Late-onset SLE has a poor long-term prognosis. Infection is the major cause of death in patients with late-onset lupus. Disease activity, medical care, and onset age are strongly related to death of late-onset SLE. [ABSTRACT FROM AUTHOR]

: The clinical manifestations, treatment and course, and articular outcomes of 24 children with juvenile-onset Still’s disease (JOSD) and 21 adults with adult-onset Still’s disease (AOSD) were compared retrospectively. There was no significant difference in the initial clinical and laboratory manifestations except that more adults presented with a sore throat (81% vs 46%, p = 0.03). Although serum ferritin was almost always elevated in both diseases, adults had significantly higher serum ferritin concentrations compared with those of children. Steroid treatment was required in 71% of children and 52% of adults, while disease-modifying antirheumatic drugs were used in 42% of children and 24% of adults during the course. Chronic arthritis (>6 months) occurred in comparable proportions of patients with JOSD and AOSD (46% vs 38%, p = 0.82), irrespective of the disease pattern (monocyclic or polycyclic). However, severe deforming arthritis with marked functional limitation occurred only in JOSD, particularly with polyarthritis at disease onset (more than five affected joints). In contrast, AOSD patients with chronic arthritis had a favourable functional outcome at the end of the follow-up. Our study suggested different articular outcomes of Still’s disease in Chinese children and adults. [ABSTRACT FROM AUTHOR]

This paper aims to study the survival and risk factors affecting the long-term prognosis of Chinese patients with systemic lupus erythematosus (SLE). We collected clinical data of 1,072 SLE patients at the time of diagnosis. The Kaplan-Meier method was used to estimate the survival rate, and the Cox proportional hazard regression model for the risk factors affecting prognosis. Of the original 1,072 recruited SLE patients, 665 (570 females and 95 males) were successfully followed up. Mean follow-up was 5.47 ± 4.62 years. Mean age of onset was 29.4 ± 13.4 years. Eighty-one patients did not survive during follow-up; infection, followed by cardiovascular disease, renal failure and SLE disease activity were the leading causes of death. The 5- and 10-year survival rates were 91.2 and 79.6 %, respectively. Moreover, the 5-year survival rates of female and male patients were 92.6 and 81.6 % respectively, and the 10-year survival rates were 80.8 and 62.3 %, respectively. Univariate analyses indicated that male gender, older age of onset, hypertension, increased blood creatinine levels, and high-density lipoprotein cholesterol at the time of diagnosis of SLE were risk factors for all-cause mortality. After adjusting for potential confounders by multivariate analysis, male gender, older age of onset, and high SLEDAI scores at the time of diagnosis were independent risk factors for all-cause mortality in SLE patients. The long-term survival of Chinese SLE patients is comparable to that of other countries. Older age of onset, high disease activity, and decline in renal function are independent risk factors for mortality in patients with SLE.