Rheumatology Advances in Practice最新文献

{"title":"Challenges in the transition of care for rare connective tissue diseases: results from the 2023 ERN ReCONNET Transition of Care Task Force survey.","authors":"Edoardo Marrani, Mojca Zajc Avramovic, Diana Marinello, Rosaria Talarico, Chiara Baldini, Eva Collado-González, João Eurico Fonseca, Linda Schraven, Filipa Oliveira Ramos, Paola Triggianese, Arjan Vissink, Marta Mosca, Tadej Avcin, Gabriele Simonini","doi":"10.1093/rap/rkae149","DOIUrl":"10.1093/rap/rkae149","url":null,"abstract":"<p><strong>Objectives: </strong>Two different European Reference Networks cover CTDs with paediatric onset, the European Reference Network on Rare and Complex Connective Tissue Diseases (ERN ReCONNET) and the European Reference Network on Rare Immunological Disorders (ERN RITA). The transition of care is a significant focus, with ReCONNET centres actively addressing this through updated programs. Despite these efforts, challenges persist. We aimed to inventory transitional care programs for rare CTDs across Europe.</p><p><strong>Methods: </strong>In April 2023, the ERN ReCONNET Transition of Care Task Force, consisting of expert clinicians, patient advocates and coordination team members, created a survey to assess transitional care practices. The survey was distributed to ERN ReCONNET and ERN RITA centres and responses received by 15 March 2024 were analysed.</p><p><strong>Results: </strong>A total of 67 responses from 59 centres across 20 European countries were collected. Paediatric rheumatologists typically initiated the transition process (49% of centres). Twenty centres had joint clinics. Despite positive self-assessments of transitional programs, significant limitations were noted. Transition policies varied, with only 40% of centres having a formal standardized policy and less than half of the centres adhering to available transition of care guidelines. Transfer readiness was evaluated using validated questionnaires in 13% of centres, while 29% transitioned patients based solely on age without any readiness assessments. The main challenges included finding adult-oriented centres and the lack of guidelines or engagement from adult centres. Adult healthcare providers also noted a lack of training in adolescent medicine.</p><p><strong>Conclusion: </strong>The survey highlighted diverse transition practices and resources across centres, with challenges in readiness evaluation and the use of guidelines. Despite these obstacles, respondents rated ongoing transition processes positively. Enhancing patient perspectives in the transition process is crucial to meet their needs during this critical phase.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae149"},"PeriodicalIF":2.1,"publicationDate":"2025-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11780841/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143067872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating antiphospholipid syndrome: from personalized therapies to cutting-edge research.","authors":"Karen Kortright-Maldonado, Bruno Eduardo Reyes-Torres, Lilian Stephany Cabrera-Lopez, Pedro Rodríguez-Henríquez, Erika Karina Tenorio-Aguirre, Froylan D Martínez-Sánchez","doi":"10.1093/rap/rkaf005","DOIUrl":"10.1093/rap/rkaf005","url":null,"abstract":"<p><p>APS is an autoimmune disorder characterized by thrombosis and pregnancy complications, primarily driven by aPLs such as LA, aCL and anti-β2 glycoprotein I (a-β2GPI). Despite advances in anticoagulation therapies, managing refractory APS cases remains challenging. Emerging therapies, including rituximab, eculizumab and HCQ, show potential in addressing the underlying mechanisms of APS. Additionally, research into genetic and environmental factors, particularly the gut microbiome's role through molecular mimicry, suggests new therapeutic pathways. Diagnostic advancements, such as the adjusted Global Antiphospholipid Syndrome Score (aGAPSS), metabolomic profiling and MRI, have improved risk stratification and early detection. Non-traditional biomarkers like anti-phosphatidylserine/prothrombin (aPS/PT) and anti-Domain I antibodies further enhance risk assessment. Future research should aim to validate these approaches, optimizing patient outcomes and minimizing long-term APS complications.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkaf005"},"PeriodicalIF":2.1,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751690/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024605","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of disease manifestations on first biologic drug survival in axial spondyloarthritis: a real-life Canadian study.","authors":"Raphaël Hurtubise, Sherry Rohekar, Nigil Haroon, Zeynep Baskurt, Tina Chim, Michel Zummer, Robert D Inman, Nicolas Richard","doi":"10.1093/rap/rkaf004","DOIUrl":"10.1093/rap/rkaf004","url":null,"abstract":"<p><strong>Objectives: </strong>The primary objective of this study was to assess the impact of extramusculoskeletal manifestations (EMMs) and peripheral musculoskeletal features on first biologic drug survival in subjects with axial spondyloarthritis (axSpA). The secondary objective was to evaluate the impact of reasons for treatment discontinuation.</p><p><strong>Methods: </strong>A total of 593 axSpA patients from the SpondyloArthritis Research Consortium of Canada initiating a first biologic drug were identified between 2003 and 2023. Drug survival was presented using Kaplan-Meier curves for each disease manifestation and compared using the logrank test. A Cox proportional hazards model was used to analyse independent predictors of discontinuation. The impact of reasons for treatment discontinuation was assessed using a competing risk analysis.</p><p><strong>Results: </strong>The presence of psoriasis, nail psoriasis, dactylitis, at least one EMM or at least one peripheral musculoskeletal manifestation was associated with prolonged drug survival compared with subjects without these disease manifestations. In multivariable analysis, psoriasis [hazard ratio (HR) 0.53 (95% CI 0.33, 0.86)] and at least one peripheral musculoskeletal manifestation [HR 0.65 (95% CI 0.47, 0.92)] were independently associated with a lower risk for biologic discontinuation. The presence of psoriasis or dactylitis was associated with reduced treatment discontinuation in patients who stopped their biologic medication for ineffectiveness but not when treatment was discontinued due to adverse events.</p><p><strong>Conclusions: </strong>This study showed that the presence of some axSpA disease manifestations were associated with prolonged biologic drug survival. Psoriasis and peripheral musculoskeletal manifestations were the most significant predictors of treatment retention. Future research will be needed to further refine treatment strategies according to specific disease manifestations.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkaf004"},"PeriodicalIF":2.1,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11780884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143067875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Kawasaki disease preceded by acute appendicitis treated with intravenous immunoglobulin without surgery.","authors":"Megumi Sato, Yuji Fujita, Junko Naganuma, Kaori Sekine, Shun Watanabe, Kei Ogino, Kan Suzuki, Shigemi Yoshihara","doi":"10.1093/rap/rkaf003","DOIUrl":"10.1093/rap/rkaf003","url":null,"abstract":"<p><p>Graphical Abstract.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkaf003"},"PeriodicalIF":2.1,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751686/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to: OA20 Differentiating Lupus Nephritis from Pre-eclampsia-A Diagnostic Dilemma.","authors":"","doi":"10.1093/rap/rkae156","DOIUrl":"https://doi.org/10.1093/rap/rkae156","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1093/rap/rkae117.020.].</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae156"},"PeriodicalIF":2.1,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11700574/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Determinants of physical function, as measured using PROMIS PF-10a, in patients with rheumatoid arthritis: results from the international COVID-19 Vaccination in Autoimmune Diseases (COVAD) study.","authors":"Saadia Sasha Ali, Christiana Demetriou, Ioannis Parodis, Ai Lyn Tan, Abraham Edgar Gracia-Ramos, Mrudula Joshi, Carlo V Caballero-Uribe, Sreoshy Saha, James B Lilleker, Arvind Nune, John D Pauling, Chris Wincup, Kshitij Jagtap, Dzifa Dey, Marcin Milchert, Oliver Distler, Hector Chinoy, Vikas Agarwal, Latika Gupta, Elena Nikiphorou","doi":"10.1093/rap/rkae154","DOIUrl":"10.1093/rap/rkae154","url":null,"abstract":"<p><strong>Objectives: </strong>Physical function in RA is largely influenced by multiple clinical factors, however, there is a growing body of evidence that psychological state and other comorbidities also play an essential role. Using data obtained in the COVID-19 Vaccination in Autoimmune Diseases study, an international self-reported e-survey, we aimed to explore the predictive ability of sociodemographic and clinical variables on Patient-Reported Outcomes Measurement Information System Physical Function Short Form 10a (PROMIS PF-10a) in RA and to investigate variation in disease activity and functional outcomes based on country-level socio-economic parameters.</p><p><strong>Methods: </strong>Patient demographics, disease characteristics including current symptom status, functional status and treatment variables, as well as income level of the country of residence, were extracted from survey responses. PROMIS PF-10a scores were compared across country income levels. The influence of extracted variables on reversed PROMIS PF-10a scores were investigated using negative binomial univariable- and multivariable regression.</p><p><strong>Results: </strong>A total of 1342 RA patients were included in this analysis. In the optimised parsimonious predictive model for reversed PROMIS PF-10a, older age, female gender, disease duration, fatigue and pain levels were independently associated with worse physical function, whereas Asian ethnicity, higher overall physical health ratings, ability to carry out everyday activities and residing in a country with an upper-middle or high-income level were independently associated with better physical function.</p><p><strong>Conclusion: </strong>Our study highlights that clinical factors remain strong predictors of physical function in RA, irrespective of individual and country-level socio-economic differences. Interestingly, high country-level income was associated with better physical function, irrespective of individual sociodemographic and clinical factors.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae154"},"PeriodicalIF":2.1,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rapidly progressive digital necrosis in polyarteritis nodosa.","authors":"Soichiro Kubota, Yoshiaki Kobayashi, Shunichiro Hanai, Daiki Nakagomi","doi":"10.1093/rap/rkae153","DOIUrl":"https://doi.org/10.1093/rap/rkae153","url":null,"abstract":"","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae153"},"PeriodicalIF":2.1,"publicationDate":"2024-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11709451/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142954217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Scleroderma and Raynaud's UK (SRUK) national survey to explore rheumatologists' awareness, approaches to diagnosis and management and training needs within systemic sclerosis.","authors":"Emma Blamont, Sue Farrington, Nina Glover-Southworth, Gemma Cornwell, Laura Gibson, Melanie Sloan, Michael Hughes","doi":"10.1093/rap/rkae152","DOIUrl":"10.1093/rap/rkae152","url":null,"abstract":"<p><strong>Objectives: </strong>Patients' outcomes and experiences can be affected by rheumatologist knowledge and awareness of systemic sclerosis (SSc). Our survey, directed at UK-based rheumatologists, aimed to expand our understanding of the above points along with their ability to keep up to date with guidelines defining best practice.</p><p><strong>Methods: </strong>Rheumatologists were invited to participate in an online survey, with the results analysed and presented descriptively and graphically by SRUK.</p><p><strong>Results: </strong>Of the 150 UK rheumatologists surveyed, 2% reported that they had not heard of SSC and 18.7% reported having a limited understanding of the condition. A total of 44% of respondents reported that they were fully versed in the signs and symptoms of SSc. The majority of those surveyed shared the broad view that all aspects of SSc, including services (63.4%), specialist positions (64.7%), research (73.1%) and training and education (70%), are either completely or somewhat underfunded. Most respondents (87.4%) reported that their workload allowed them to 'somewhat' (48.74%) or 'completely' (38.66%) keep up to date with official guidelines. Scleroderma and Raynaud's UK (SRUK) (47.9%), followed by NICE (43.7%), were the most highly used sources of information utilized among those surveyed.</p><p><strong>Conclusion: </strong>Our survey reveals a serious gap in the awareness and the signs and symptoms of SSc among some UK rheumatologists, in addition to a perception that services, training and education in this area are underfunded. Our findings indicate that there is a role for the provision of further education and training as part of continued professional development.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae152"},"PeriodicalIF":2.1,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11739612/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143010859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Analysis of risk factors for abnormal renal function in patients with gout in Southwest China: a cross-sectional study.","authors":"Ting Zhang, Ziqian Zeng, Dan Xu, Wantai Dang","doi":"10.1093/rap/rkae151","DOIUrl":"https://doi.org/10.1093/rap/rkae151","url":null,"abstract":"<p><strong>Objective: </strong>To analyse the associations between renal function and clinical laboratory indicators and explore the renal function abnormality risk factors for gout patients in Southwest China.</p><p><strong>Methods: </strong>Outpatient and hospitalized gout patients (<i>n</i> = 4384) at the First Affiliated Hospital of Chengdu Medical College between January 2017 and December 2020 were divided into normal (<i>n</i> = 2393) and abnormal (<i>n</i> = 1991) renal function groups according to their eGFR. The relationships between clinical laboratory indicators and the eGFR were analysed, and a logistic regression model was fit to identify significant risk factors.</p><p><strong>Results: </strong>Sex, age, absolute lymphocyte count (ALC), cystatin C (CysC), homocysteine (Hcy) and thyroid stimulating hormone (TSH) were associated with renal function abnormalities (<i>P</i> < 0.05), whereas age [odds ratio (95% CI) = 1.06 (1.05-1.08), <i>P</i> < 0.001], Hcy [1.02 (1.00-1.04), <i>P</i> = 0.028], CysC [1.72 (1.54-1.92), <i>P</i> < 0.001], ALC [0.71 (0.52-0.97), <i>P</i> = 0.03] and TSH [1.08 (1.00-1.17), <i>P</i> = 0.049] were abnormal renal function risk factors for gout patients. After stratification by UA, binary logistic regression analysis identified the following risk factors: Q1 age [1.06 (1.02-1.11), <i>P</i> = 0.003], CysC [1.67 (1.30-2.16), <i>P</i> < 0.001]; Q2 age [1.09 (1.06-1.12), <i>P</i> < 0.001], CysC [1.55 (1.28-1.88), <i>P</i> < 0.001], FT3 [0.66 (0.46-0.96), <i>P</i> = 0.029]; Q3 age [1.06 (1.03-1.09), <i>P</i> < 0.001], CysC [1.75 (1.41-2.18), <i>P</i> < 0.001], Hcy [1.04 (1.00-1.08), <i>P</i> = 0.047], ALC [0.35 (0.18-0.69), <i>P</i> = 0.002]; Q4 age [1.05 (1.02-1.09), <i>P</i> = 0.004], CysC [1.79 (1.40-2.30), <i>P</i> < 0.001].</p><p><strong>Conclusion: </strong>ALC and levels of TSH and serum Cys could be used for monitoring for abnormal renal function in patients with gout.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae151"},"PeriodicalIF":2.1,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11696702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The pattern of anti-IL-6 versus non-anti-IL-6 biologic disease modifying anti-rheumatic drugs use in patients with rheumatoid arthritis in Wales, UK: a real-world study using electronic health records.","authors":"Roxanne Cooksey, Jonathan Kennedy, Muhammad Rahman, Sinead Brophy, Ernest Choy","doi":"10.1093/rap/rkae140","DOIUrl":"10.1093/rap/rkae140","url":null,"abstract":"<p><strong>Objective: </strong>Investigating factors associated with drug initiation and discontinuation in patients treated with anti-IL-6 biologic DMARDs (bDMARDs) (tocilizumab or sarilumab) <i>vs</i> non-anti-IL-6 (anti-TNF, B or T cell therapies) bDMARDs for RA.</p><p><strong>Methods: </strong>A retrospective cohort study of patients with the diagnosis of RA in the Secure Anonymised Information Linkage Databank, comprising primary and secondary care and specialist rheumatology clinic records for >90% of the population in Wales, UK. Patients initiated on first bDMARD treatment, discontinuation and clinical outcomes including infection and hospitalisation were analysed using Cox regression analysis.</p><p><strong>Results: </strong>Of patients identified with RA in their primary care records, 95.7% (4691/4922) received conventional synthetic DMARDs (csDMARDs). More than one-third (36.2%) were treated with bDMARDs (1784/4922). Of these biologic-naïve patients, 6.5% (116) were treated with anti-IL-6 bDMARDs; this treatment was associated with a previous history of infection [difference 8.8% (95% CI 1.1, 17.8)] and kidney disease [14.3% (95% CI 8.0, 22.5)]. Treatment discontinuation was significantly higher in the non-anti-IL-6 bDMARD-treated patients (23.1%) compared with the anti-IL-6 bDMARD-treated individuals (18.1%) [difference 9.4% (95% CI 1.1, 15.7)]. For those discontinuing a first line of treatment, 385 patients (23%) and 21 patients (18%) switched to an alternative bDMARD from the non-anti-IL-6 and anti-IL-6 groups, respectively.</p><p><strong>Conclusion: </strong>Comorbidities, history of infection and kidney disease were associated with choosing anti-IL-6 bDMARDs in biologic-naïve RA patients in Wales. Anti-IL-6 bDMARD-treated biologic-naïve patients were more likely to continue treatment than non-IL-6 bDMARD-treated patients.</p>","PeriodicalId":21350,"journal":{"name":"Rheumatology Advances in Practice","volume":"9 1","pages":"rkae140"},"PeriodicalIF":2.1,"publicationDate":"2024-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11651880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142855130","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}