Original research on Behçet's syndrome: a bibliometric analysis over 20 years (2000-2019).

OBJECTIVES: We aimed to perform a bibliometric analysis of original research articles on Behçet's syndrome (BS) published over the last 20 years prior to the COVID-19 pandemic, and to systematically describe their characteristics and citation records. METHODS: The PubMed database was searched for any article published on BS between 2000 and 2019. We identified all original research articles and categorised them by country of origin and type of research, i.e., clinical, translational and basic. Each article's impact was assessed using the individual citation numbers from Google Scholar search engine; we also calculated the median annual citation rates (ACRs), both per country and research type. RESULTS: Of a total of 2,381 retrieved original articles from 51 countries, the majority reported on clinical (52.6%), followed by translational (46.0%) and basic research (1.4%). Turkey had the highest number of publications (39% of articles) followed by four countries (Korea, China, Japan, Italy) where BS is also relatively prevalent. However, regarding median ACRs, France was first, followed by the United Kingdom, Germany and Collaboration. Although the number of articles has almost doubled between 2010-2019 versus 2000-2009, median ACRs across either clinical or translational research had a downwards trend. CONCLUSIONS: Researchers from countries where BS is prevalent are more productive, albeit their work is of lower impact compared to countries with generally higher research budgets. A considerable increase of original research articles on BS is observed over time but further funding may be warranted for a parallel increase in the respective scientific impact.

New-Onset MDA-5 Dermatomyositis in a Patient Following COVID-19 Vaccination: A Case Report.

Vaccination against Sars-CoV-2 has been proven to significantly reduce COVID-19 morbidity and mortality and is therefore recommended for the general population, and especially for seniors with impaired immunity. However, it is currently postulated that COVID-19 vaccines could rarely induce autoimmune diseases in previously healthy individuals. We report a case of new-onset anti-melanoma differentiation-associated protein 5 (anti-MDA5) antibody-positive dermatomyositis in a patient presenting with rash and fever following the third dose of COVID-19 vaccine. The laboratory testing revealed high titres of anti-MDA-5 antibody and chest computed tomography showed micronodular lesions and ground glass opacities bilaterally. The patient was promptly treated with corticosteroids, methotrexate, and azathioprine, and was later started on rituximab due to dermatomyositis rash exacerbation along with newly formed, diffuse skin ulcers. Our case highlights the potential immunogenicity of COVID-19 vaccines and the need for further reporting of rare rheumatic syndromes possibly related to COVID-19 disease and vaccination.

Prevalence and target attainment of traditional cardiovascular risk factors in patients with systemic lupus erythematosus: a cross-sectional study including 3401 individuals from 24 countries.

BACKGROUND: Systemic lupus erythematosus (SLE) is characterised by increased cardiovascular morbidity and mortality risk. We aimed to examine the prevalence of traditional cardiovascular risk factors and their control in an international survey of patients with systemic lupus erythematosus. METHODS: In this multicentre, cross-sectional study, cardiovascular risk factor data from medical files of adult patients (aged ≥18) with SLE followed between Jan 1, 2015, and Jan 1, 2020, were collected from 24 countries, across five continents. We assessed the prevalence and target attainment of cardiovascular risk factors and examined potential differences by country income level and antiphospholipid syndrome coexistence. We used the Systemic Coronary Risk Evaluation algorithm for cardiovascular risk estimation, and the European Society of Cardiology guidelines for assessing cardiovascular risk factor target attainment. People with lived experience were not involved in the research or writing process. FINDINGS: 3401 patients with SLE were included in the study. The median age was 43·0 years (IQR 33-54), 3047 (89·7%) of 3396 patients were women, 349 (10.3%) were men, and 1629 (48·1%) of 3390 were White. 556 (20·7%) of 2681 patients had concomitant antiphospholipid syndrome. We found a high cardiovascular risk factor prevalence (hypertension 1210 [35·6%] of 3398 patients, obesity 751 [23·7%] of 3169 patients, and hyperlipidaemia 650 [19·8%] of 3279 patients), and suboptimal control of modifiable cardiovascular risk factors (blood pressure [target of <130/80 mm Hg], BMI, and lipids) in the entire SLE group. Higher prevalence of cardiovascular risk factors but a better blood pressure (target of <130/80 mm Hg; 54·9% [1170 of 2132 patients] vs 46·8% [519 of 1109 patients]; p<0·0001), and lipid control (75·0% [895 of 1194 patients] vs 51·4% [386 of 751 patients], p<0·0001 for high-density lipoprotein [HDL]; 66·4% [769 of 1158 patients] vs 60·8% [453 of 745 patients], p=0·013 for non-HDL; 80·9% [1017 of 1257 patients] vs 61·4% [486 of 792 patients], p<0·0001 for triglycerides]) was observed in patients from high-income versus those from middle-income countries. Patients with SLE with antiphospholipid syndrome had a higher prevalence of modifiable cardiovascular risk factors, and significantly lower attainment of BMI and lipid targets (for low-density lipoprotein and non-HDL) than patients with SLE without antiphospholipid syndrome. INTERPRETATION: High prevalence and inadequate cardiovascular risk factor control were observed in a large multicentre and multiethnic SLE cohort, especially among patients from middle-income compared with high-income countries and among those with coexistent antiphospholipid syndrome. Increased awareness of cardiovascular disease risk in SLE, especially in the above subgroups, is urgently warranted. FUNDING: None.

Metabolic syndrome in antiphospholipid syndrome versus rheumatoid arthritis and diabetes mellitus: Association with arterial thrombosis, cardiovascular risk biomarkers, physical activity, and coronary atherosclerotic plaques.

Background: Cardiovascular disease (CVD) is the foremost cause of morbidity and deaths in antiphospholipid syndrome (APS), driven by thrombo-inflammation and atherothrombosis mechanisms. Metabolic syndrome (MetS) is a proinflammatory and prothrombotic state characterized by increased CVD risk. We aimed to evaluate the prevalence of MetS in APS patients compared to rheumatoid arthritis (RA) and diabetes mellitus (DM) and its associations with clinical and laboratory patient characteristics and vascular ultrasound (US) markers of subclinical atherosclerosis. Methods: We included 414 patients in our study: 138 patients with APS (median age: 44.9 years, females 70%) and matched 1:1 for age and sex RA and DM subjects. Three sets of criteria were used for MetS diagnosis: Joint Interim Statement (JIS), International Diabetes Federation (IDF) and modified National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII). The demographic, clinical and laboratory characteristics of all participants were recorded and carotid and femoral US was performed in patients with APS. Multivariate regression models were applied. Results: Prevalence of MetS was 23.9%, 23.2%, 20.3% (based on JIS, IDF, modified NCEP-ATPIII criteria, respectively) in APS versus 17.4%, 17.4%, 13% in RA (p=0.181, p=0.231, p=0.106, respectively), and 44.2%, 44.2%, 40.6% in DM patients. In multivariate analysis, patients with systemic lupus erythematosus- related APS had an approximately 2.5-fold higher risk of MetS versus RA patients. MetS in APS was independently associated with arterial thrombosis (Odds ratio 3.5, p=0.030). Odds ratio for MetS was 1.16 for each one unit increase in C-reactive protein levels according to JIS and IDF criteria, and 1.49 and 1.47 for each one unit increase in uric acid levels using the IDF and modified NCEP-ATPIII models, respectively. APS patients with atherosclerotic carotid plaques had 4 to 6.5-fold increased risk of MetS. Odds for MetS were decreased by 26% with an increase in physical activity by one hour per week. Conclusions: MetS is present in approximately one-fourth of APS patients at a comparable prevalence to that observed in patients with RA. MetS in APS is associated with arterial thrombosis, cardiovascular risk biomarkers, physical activity, and subclinical atherosclerosis, supporting its role in cardiovascular risk stratification and management in APS.

Cardiovascular risk management in antiphospholipid syndrome: trends over time and comparison with rheumatoid arthritis and diabetes mellitus.

OBJECTIVE: Antiphospholipid syndrome (APS) is characterised by increased cardiovascular morbidity and mortality, related to thrombo-inflammatory and atherogenic mechanisms. We examined the achievement of traditional cardiovascular risk factor (CVRF) therapeutic goals in APS versus other high cardiovascular risk disorders such as rheumatoid arthritis (RA) and diabetes mellitus (DM), and trends over time. METHODS: 122 patients with APS (74 primary APS, female 68%, mean age 44.5±11.3) were classified according to their first visit (2011-2015 and 2016-2020 APS subgroups, 61 patients in each subgroup) and matched 1:1 for age/sex with patients with RA and DM. Cardiovascular risk was estimated by the Systemic Coronary Risk Evaluation, and the CVRF therapeutic targets were defined according to the European Society of Cardiology (ESC) guidelines. Individual and multiple CVRF control was compared between APS subgroups, and in APS versus RA and DM. RESULTS: We found a comparable or higher prevalence of CVRFs between APS and age-matched/sex-matched patients with RA and DM but low CVRF target attainment in APS according to the ESC guidelines. Despite improving trends between 2011-2015 and 2016-2020, CVRF control in high/very high-risk patients with APS was 12%, 18%, 24% and 35% for low-density lipoprotein, waist circumference, exercise and body mass index, respectively, and 59%-65% for triglycerides, high-density lipoprotein (HDL) and blood pressure, in 2016-2020 subgroup. CVRF control was worse in APS versus RA for smoking (p=0.014), HDL (p<0.001), waist circumference (p=0.042) and five CVRFs (p=0.030), and versus DM for exercise (p=0.077). Similar results were found in the sensitivity analysis. CONCLUSIONS: Comparable prevalence of modifiable CVRFs to RA and DM but suboptimal CVRF target achievement was observed in APS, especially in high/very high-risk patients, highlighting the need for CVRF management strategies.