{"title":"Efficacy and safety of upadacitinib, a selective JAK-1 inhibitor in treatment of ankylosing spondylitis: a meta-analysis.","authors":"Qi Yao, Yixuan Zhu, Yanling Ma, Yanfang Pu, Xueting Yang, Zhiqing Zhang","doi":"10.1186/s41927-025-00467-1","DOIUrl":"10.1186/s41927-025-00467-1","url":null,"abstract":"<p><strong>Objective: </strong>To systemically assess efficacy and safety of upadacitinib (UPA), a selective inhibitor of Janus kinase 1 (JAK1) in treatment of ankylosing spondylitis (AS).</p><p><strong>Methods: </strong>Available databases were used to retrieve literatures of randomized controlled trials (RCTs) of UPA for AS treatment until February 2024. After that, the data were extracted and the Revman 5.4 software was used to conduct a meta-analysis.</p><p><strong>Results: </strong>A total of 6 articles and 1653 patients (920 in a UPA group (15 mg, q.d) and 733 in a placebo group) were selected in this study. Respectively, UPA treatment significantly increased numbers of the AS patients having 40%, 20%, or partial remission (PR) improvement in assessment of spondylo arthritis international society (ASAS) (ASAS 40: 95%CI: 2.41-4.3, p < 0.00001; ASAS 20: 95%CI: 2.12-3.62, p < 0.00001; ASAS PR: 95%CI: 2.81-7.48, p < 0.00001), Bath ankylosing spondylitis disease activity index (BASDAI50) (95%CI: 2.28 ~ 4.10, p < 0.00001), quality of life (95%CI: 2.06 ~ 3.17, p < 0.00001), AS disease activity score low disease activity (ASDAS LDA) (95%CI: 3.07~9.96, p < 0.00001), ASDAS inactive disease (ID) (95%CI: 2.03 ~ 17.22, p = 0.001), short-form 36 physical component summary (SF-36PCS) (95%CI: 1.53 ~2.81, p < 0.00001), and markedly reduced ASDAS C-reactive protein (CRP) (95%CI: -1.22 ~ -0.42, p < 0.0001), total back pain score (95%CI: -2.01 ~ -0.51, p = 0.001), nighttime back pain score (95%CI: -1.96 ~ -0.54, p = 0.0006), spondylo arthritis research consortium of Canada magnetic resonance imaging (SPARCC MRI) spine score (95%CI: -7.78--3.50, p < 0.00001) and SPARCC MRI sacroiliac joint score (95%CI: -5.99 - -3.09, p < 0.00001), Bath ankylosing spondylitis function index (BASFI) score (95%CI: -1.45 ~ -0.81, p < 0.00001), Maastricht ankylosing spondylitis enthesitis score (MASES) (95%CI: -2.34~-0.35, p = 0.008). Except for neutropenia (95%CI: 1.25 ~ 15.60, p = 0.02), no other adverse effects (AEs) were significantly different between the UPA treatment and placebo.</p><p><strong>Conclusions: </strong>Through a literature analysis, it reveals that UPA offers significant therapeutic benefits to AS patients with a relatively high safety profile.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"19"},"PeriodicalIF":2.1,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11834275/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448130","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}
BMC RheumatologyPub Date : 2025-02-18DOI: 10.1186/s41927-025-00463-5
Neva Pfyl, Lea Ettlin, Karin Niedermann, Anne-Kathrin Rausch
{"title":"Evaluation of usability, feasibility and acceptance of the digital training diary Trainingslog for individuals with axSpA: a mixed-method study.","authors":"Neva Pfyl, Lea Ettlin, Karin Niedermann, Anne-Kathrin Rausch","doi":"10.1186/s41927-025-00463-5","DOIUrl":"10.1186/s41927-025-00463-5","url":null,"abstract":"<p><strong>Background: </strong>Physical activity (PA), including regular exercise, is essential for the successful management of axial spondyloarthritis (axSpA). To promote a physically active lifestyle, a digital training diary (Trainingslog) was developed in an user-centered approach by the Swiss Ankylosing Spondylitis Association (SVMB). A training diary promotes PA through feedback, goal setting and self-monitoring, which can also be used for PA counselling by physiotherapists (PT). Usability, feasibility and acceptance are essential for the successful implementation of a mobile Health Intervention such as the Trainingslog. The study objective is to evaluate the usability, feasibility and acceptance of the Trainingslog for individuals with axSpA and PTs.</p><p><strong>Methods: </strong>A mixed-methods design was performed among potential end-users of the Trainingslog. Quantitative data was collected by use of questionnaires (System Usability Scale (SUS, 0-100 scale), user version of the Mobile App Rating Scale (uMARS, 5 point scale)) and number of training entries. Subsequently, qualitative data was gathered through semi-structured online focus groups or individual interviews.</p><p><strong>Results: </strong>11 PTs (9 women, mean age 52.5 [SD 15.6]) and 10 individuals with axSpA (6 women, mean age 48 [SD 13.4]) participated. The quantitative data showed mean SUS scores for usability of 82.5 [SD 21.76] for PTs and 77.0 [SD 9.34] for individuals with axSpA. The mean uMARS sector B scores for feasibility were 4.2 [SD 0.49] for PTs and 4.1 [SD 0.38] for individuals with axSpA. Acceptance, as indicated by the uMARS results (mean score > 3 in Sectors E and F for both groups), was given. But there was a lower-than-expected agreement in the training entries, with 59.86% of entries matching between the Trainingslog and the paper diary. The qualitative analysis unveiled that while usability and feasibility were good, acceptance was lower, primarily due to the use of a web-based link instead of an app version.</p><p><strong>Conclusion: </strong>The Trainingslog showed a good usability and feasibility, while the acceptance was lower than expected. Acceptance could be increased by offering the Trainingslog as an app-based version, along with implementing additional recommendations for enhancement. Consequently, the Trainingslog has the potential to be applied in PA counselling by PTs or as a self-monitoring tool for individuals with axSpA.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"18"},"PeriodicalIF":2.1,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11834626/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448131","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":"Psoriatic arthritis in Jordan: a cross-sectional study of disease characteristics, patient-reported outcomes, and disease activity.","authors":"Fatima Alnaimat, Khaldoon Alawneh, Ayman AbuHelal, Omar Hamdan, Almothana Alelaimat, Manal Al Mashaleh, Ausaylah Burqan, Wala Rababah, Rabaa Rababah, Marwan Adwan","doi":"10.1186/s41927-025-00468-0","DOIUrl":"10.1186/s41927-025-00468-0","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PsA) is a chronic, inflammatory rheumatic disease. We aim to describe the characteristics of PsA patients and examine factors affecting their psychological and physical well-being.</p><p><strong>Methods: </strong>This multicenter, cross-sectional study enrolled consecutive PsA patients from rheumatology clinics over six months. Data was collected through questionnaires and chart reviews. Disease activity was assessed using Disease Activity in Psoriatic Arthritis (DAPSA) and Psoriatic Arthritis Impact of Disease-12 (PsAID-12), with fibromyalgia and psychological well-being screened via Fibromyalgia Rapid Screening Tool (FiRST) and Patient Health Questionnaire-4 (PHQ-4), respectively.</p><p><strong>Results: </strong>The study enrolled 105 patients with a mean age of 45.6 ± 12.9, and 46.7% (N = 49) were males. The predominant disease type was polyarthritis (80%, N = 84), with 90.5% (N = 95) having psoriasis (PSO). Arthritis and PSO were diagnosed simultaneously in 18 patients (17.1%), arthritis preceded PSO in 11 patients (10.5%) by 3.5 ± 3.8 years, and PSO preceded arthritis in 76 patients (72.4%) by 10.65 ± 11.27 years. The diagnostic delay of PsA was 3.1 ± 4.9 years. Methotrexate was used by 50.5% (N = 53) and 20% (N = 21) used anti-TNF. Severe disease activity, according to DAPSA scores, was present in 38.1%, positive screening for fibromyalgia in 29.5% (N = 31), and 35.2% (N = 37) had severe depression and anxiety-related symptoms. Using multivariate regression analysis, Obesity (OR = 3.267, 95% CI: 1.015-10.513) and the presence of CVD (OR = 4.769, 95% CI: 1.121-20.293) were predictors of bone erosions. PsAID-12 scores and ESR were associated with severe depression and anxiety-related symptoms (95% OR: 1.443-4.459 and 1.001:1.078), respectively.</p><p><strong>Conclusions: </strong>Patients with PsA often face diagnostic delays, with fibromyalgia, depression, and anxiety being common, resulting in poorer patient-reported outcomes.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"16"},"PeriodicalIF":2.1,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11831762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143439887","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}
BMC RheumatologyPub Date : 2025-02-17DOI: 10.1186/s41927-025-00464-4
Katie L Druce, Yumna Masood, Helen Chadwick, Sarah Skyrme, Deb Griffiths-Jones, Ramiro D Bravo Santisteban, Peter Bower, Jill Firth, Charlotte A Sharp, Christopher J Armitage, Dawn Dowding, John McBeth, Caroline Sanders, William G Dixon, Sabine N van der Veer
{"title":"Preparing to deliver a stepped wedge cluster-randomised trial to test the effectiveness of daily symptom tracking integrated into electronic health records for managing rheumatoid arthritis: a mixed-methods feasibility trial.","authors":"Katie L Druce, Yumna Masood, Helen Chadwick, Sarah Skyrme, Deb Griffiths-Jones, Ramiro D Bravo Santisteban, Peter Bower, Jill Firth, Charlotte A Sharp, Christopher J Armitage, Dawn Dowding, John McBeth, Caroline Sanders, William G Dixon, Sabine N van der Veer","doi":"10.1186/s41927-025-00464-4","DOIUrl":"10.1186/s41927-025-00464-4","url":null,"abstract":"<p><strong>Background: </strong>We sought to assess the feasibility of a stepped-wedge cluster-randomised trial testing the effectiveness of a complex mHealth intervention called REMORA: a co-designed smartphone app enabling daily, weekly and monthly symptom tracking integrated into electronic health records for people with rheumatoid arthritis (RA).</p><p><strong>Methods: </strong>We conducted a mixed-methods feasibility trial using a convergent approach with some explanatory sequential elements. Patients were eligible to take part if they were older than ≥18 years of age, had (suspected) RA or undifferentiated inflammatory arthritis, and consented to take part from two outpatient departments. We analysed quantitative app and electronic health record data descriptively. We analysed qualitative data from interviews and clinic observations thematically. We assessed four feasibility domains: recruitment and consent (target: 15 patients per site), intervention uptake (≥70% of recruited participants completed on-boarding, i.e., registered with the app and submitted at least one symptom report), intervention adherence (>50% daily symptom reports provided), and measuring disease activity as the primary outcome (scores available for ≥80% of people with a follow-up clinic visit). Due to time constraints, we only recruited patients to the intervention group, leaving us unable to test the logistics of randomising sites in accordance with the trial's cluster stepped wedge design.</p><p><strong>Results: </strong>Of 130 people screened, 52 consented. Of those, 32 (62%) completed on-boarding. On-boarded participants provided symptom data on 2384/3771 (63%) of possible days. Among the 48 people who had ≥1 follow-up appointment, at least one disease activity scored was obtained for 46 (96%) of them. Factors related to intervention uptake formed the biggest threat to trial feasibility, including lack of clarity of communication and guidance, access to technology, and personal challenges (e.g., being busy or unwell).</p><p><strong>Conclusion: </strong>We found that delivering a trial to test the effectiveness of integrated symptom tracking in rheumatology outpatient settings was feasible. The future REMORA trial will contribute to the much-needed evidence base for the impact of integrated symptom tracking on care delivery and patient outcomes, including decision-making, patient experience, disease activity, and symptom burden.</p><p><strong>Trial registration: </strong>This feasibility trial was registered at https://www.isrctn.com/ on 23-Jan-2023 (ISRCTN21226438).</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"17"},"PeriodicalIF":2.1,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11834673/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143439820","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":"Are the clinical phenotypes of systemic sclerosis determinant for osteoporosis and fragility fractures?","authors":"Irene Carlino, Antonella Al Refaie, Caterina Mondillo, Giulio Manzana, Stefania Bisogno, Serena Pierguidi, Matteo Capecchi, Stefano Gonnelli, Luigi Gennari, Carla Caffarelli","doi":"10.1186/s41927-025-00462-6","DOIUrl":"10.1186/s41927-025-00462-6","url":null,"abstract":"<p><strong>Aim: </strong>Systemic sclerosis (SSc) is associated with an increased risk of osteoporosis and fractures. The aim of this single-center cross-sectional study was to evaluate whether clinical phenotype and nailfold videocapillaroscopy (NVC) pattern could influence bone mineral density (BMD) values and fragility fractures in patients with SSc.</p><p><strong>Methods: </strong>A cohort of 84 consecutive outpatients (age 63.7 ± 13.7 years) diagnosed with SSc, 43 classified as diffuse cutaneus SSc (dSSc) and 41 as limited cutaneus SSc (lSSc), were enrolled in the study. All patients underwent BMD by Dual Energy X-ray Absorptiometry (DX, pulmonary function tests for diffusing capacity of carbon monoxide (DLCO), and NVC to be assigned to an \"early,\" \"active,\" or \"late\" pattern.</p><p><strong>Results: </strong>Patients with dSSc exhibited significantly lower BMD values compared to those with lSSc; moreover, the prevalence of osteoporosis and major osteoporotic fractures were higher in dSSc than in lcSSc (39,6% and 41,9% vs. 29,2% and 34,1%, respectively). Patients with a \"late\" or \"active\" NVC pattern had a more marked reduction in BMD with respect to those with a \"early\" pattern (p < 0.05). Moreover, patients with dSSc showed a greater reduction in DLCO values compared to those with lSSc in all three capillaroscopic patterns (p < 0.05). DLCO reduction and history of previous fracture were independent predictors of total hip BMD in dSSc patients.</p><p><strong>Conclusion: </strong>Patients with SSc, and particularly those with a \"diffuse\" phenotype, have a high prevalence of osteoporosis and major osteoporotic fractures. Furthermore, in both SSc phenotypes, the presence of an \"active\" or \"late\" capillaroscopic pattern was associated with reduced BMD and DLCO values.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"15"},"PeriodicalIF":2.1,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11827457/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143424935","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}
BMC RheumatologyPub Date : 2025-02-12DOI: 10.1186/s41927-025-00465-3
Susann May, Greta Nordmann, Franziska Gabb, Katharina Boy, Magali Wagner, Niklas Ohm, Hanna Labinsky, Johannes Knitza, Sebastian Kuhn, Martin Heinze, Martin Welcker, Felix Muehlensiepen
{"title":"Between uncertainty and destiny: the patient journey in axial spondyloarthritis care from the perspectives of patients and their relatives.","authors":"Susann May, Greta Nordmann, Franziska Gabb, Katharina Boy, Magali Wagner, Niklas Ohm, Hanna Labinsky, Johannes Knitza, Sebastian Kuhn, Martin Heinze, Martin Welcker, Felix Muehlensiepen","doi":"10.1186/s41927-025-00465-3","DOIUrl":"10.1186/s41927-025-00465-3","url":null,"abstract":"<p><p>Axial spondyloarthritis (axSpA) is an inflammatory rheumatic disease primarily affecting the sacroiliac joints and spine, leading to chronic pain, fatigue, and reduced mobility. The diagnostic delay for axSpA is often long, causing significant physical, psychological, and social burdens for patients and their relatives. This study aims to explore the patient journey of individuals with axSpA and their relatives within the German healthcare system, identifying key challenges and unmet needs from symptom onset to diagnosis and treatment. A qualitative approach was employed, involving structured interviews with axSpA patients and their relatives. Participants were selected through purposive sampling to ensure diverse representation. Data collection involved individual telephone interviews, which were transcribed and analyzed using Kuckartz's structured qualitative content analysis framework. The patient journey was characterized by four distinct phases: Time before diagnosis, Diagnosis, After the diagnosis, and Current treatment. Participants reported significant psychological and emotional burdens, with many experiences attributed to chance encounters with knowledgeable healthcare professionals. Key issues included a lack of awareness among healthcare professionals, diagnostic delays, and inadequate psychological support. The perspectives of patients and their relatives highlighted the significant psychological burden they both experience throughout the journey. This underscores the need for services that cater not only to patients but also to their relatives. The study highlights critical gaps in the current healthcare system regarding the diagnosis and care of axSpA patients. To improve care, systematic efforts are needed to enhance awareness, reduce diagnostic delays, integrate psychological support, and provide comprehensive information throughout the patient journey for both, patients and relatives. Effective care should not rely on chance; systematic improvements are necessary to ensure consistent, high-quality care.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"14"},"PeriodicalIF":2.1,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11818133/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143398257","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}
BMC RheumatologyPub Date : 2025-02-07DOI: 10.1186/s41927-025-00459-1
Daniel G Fernández-Ávila, Monica Betancur, Amira Kronfly, Edwin Jáuregui
{"title":"Exploring drug utilization patterns, healthcare resource utilization, and epidemiology of rheumatoid arthritis in Colombia: a retrospective claims database study.","authors":"Daniel G Fernández-Ávila, Monica Betancur, Amira Kronfly, Edwin Jáuregui","doi":"10.1186/s41927-025-00459-1","DOIUrl":"10.1186/s41927-025-00459-1","url":null,"abstract":"<p><strong>Background: </strong>In Colombia, there is a lack of recent real- word studies that provide information on the epidemiology and healthcare resource utilization (HCRU) of rheumatoid arthritis (RA) at national level.</p><p><strong>Methods: </strong>To describe the burden of RA in Colombia, focusing on trends in drug utilization patterns, healthcare resource utilization (HCRU), and the epidemiology of adult patients diagnosed with RA between January 2017 and December 2022. This retrospective descriptive study used real-world data obtainedfrom a national claim database, SISPRO (Sistema de Información para la Protección Social). We included registries of adult patients diagnosed with RA between 2017 and 2022. We estimated the age-standardized incidence and prevalence of RA each year, drug utilization patterns for disease-modifying antirheumatic drugs (DMARDs) and glucocorticoids, rates of medical consultations, emergency room visits, and hospitalizations, and associated comorbidities and healthcare-related and pharmacy-related costs.</p><p><strong>Results: </strong>Overall, 327,430 unique patients with RA between 2017 and 2022 were included in the analysis, comprising 94,093 incident cases and 722,569 prevalent cases. The age-standardized incidence of RA ranged between 34.7 and 51.4 per 100,000 inhabitants, and the age-standardized prevalence ranged between 0.282 and 0.382 per 100 inhabitants between 2017 and 2022. The proportion of patients prescribed conventional synthetic DMARDs and biologic DMARDs decreased over the study period, from 39.23% in 2017 to 28.61% in 2021 and from 6.07% in 2017 to 3.72% in 2021, respectively. The proportion of patients prescribed targeted synthetic DMARDs increased from 0.9% in 2017 to 1.8% in 2021. The rate of medical consultations increased over the study period (from 2,406.6 in 2017 to 3,354.2 per 1,000 patients with RA in 2022). Consultation costs were the largest among all-cause annual healthcare-related costs.</p><p><strong>Conclusion: </strong>This study described the heavy burden of RA in Colombia with an increasing incidence of RA, and significant healthcare resource utilization and associated costs. Patients with RA in the country are increasingly able to access consultations with specialists and receive advanced therapies. However, there remains a need for efforts to facilitate treatment among this population. These findings emphasize the importance of tailoring RA management strategies to the local context.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"13"},"PeriodicalIF":2.1,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11803933/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143370379","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}
BMC RheumatologyPub Date : 2025-02-06DOI: 10.1186/s41927-024-00445-z
Annik Steimer, Mike O Becker
{"title":"Treatment approaches for idiopathic retroperitoneal fibrosis: a systematic review with meta-analysis.","authors":"Annik Steimer, Mike O Becker","doi":"10.1186/s41927-024-00445-z","DOIUrl":"10.1186/s41927-024-00445-z","url":null,"abstract":"<p><strong>Background: </strong>Currently, there is no standard therapy for idiopathic retroperitoneal fibrosis, so a systematic review was undertaken to assess the effectiveness of different treatment approaches.</p><p><strong>Methods: </strong>A comprehensive search of English and German literature from 1980 to 2021 was conducted using PubMed, Embase, and PreMedline. To be included, studies must have had a minimum of two patients employing the same treatment approach and reporting relevant treatment outcomes. A meta-analysis with a subgroup analysis was conducted for the primary outcomes \"regression of fibrosis,\" \"freedom from ureteric stents\" and \"relapse rate,\" and the secondary outcome \"clinical improvement.\" The lack of homogeneous data prevented a subgroup analysis for the primary outcome \"improvement in renal function.\"</p><p><strong>Results: </strong>The search resulted in a total of 3818 articles, of which 108 were selected for qualitative analysis involving a total of 1408 patients. For the meta-analysis 83 studies were included involving 1044 patients. The summary effect size of the outcomes \"regression of fibrosis,\" \"freedom from ureteric stent\" and \"clinical improvement\" was high with values between 80-97.9%. The summary relapse rate across studies was 18.1%. Subgroup analysis revealed no statistically significant differences in the effectiveness of treatment approaches for the outcomes \"regression of fibrosis\" (QM = 2.72, p = 0.74), \"freedom from ureteric stent\" (QM = 7.21, p = 0.13), \"relapse rate\" (QM = 11.34, p = 0.08) and \"clinical improvement\" (QM = 9.54, p = 0.15).</p><p><strong>Conclusions: </strong>Considering the lack of clear evidence indicating that one drug is more effective than the other, the treatment choice should depend on factors such as the potential side effects of different drug therapies, patient comorbidities, and clinician expertise. The review protocol is registered on PROSPERO under the identification number CRD42019115744.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"12"},"PeriodicalIF":2.1,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11800648/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143254824","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":"Understanding the pros and cons of spine surgery for ankylosing spondylitis: experience from a single institution study.","authors":"Mohammadreza Chehrassan, Mohammadreza Shakeri, Farshad Nikouei, Saeed Sabbaghan, Behnam Sour, Ebrahim Ameri Mahabadi, Hasan Ghandhari","doi":"10.1186/s41927-025-00461-7","DOIUrl":"10.1186/s41927-025-00461-7","url":null,"abstract":"<p><strong>Introduction: </strong>Ankylosing Spondylitis (AS) presents a complex inflammatory condition with significant impact on patients' lives. Despite advancements in understanding its pathogenesis, comprehensive elucidation remains elusive. This study investigates the outcomes of corrective spine surgery in AS patients, aiming to optimize management strategies.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted, including 28 AS patients undergoing corrective surgery from 2016 to 2021. Surgical procedures primarily targeted realignment of the spine to alleviate deformities.</p><p><strong>Results: </strong>Radiological evaluations pre- and post-operatively revealed substantial improvements in sagittal balance and kyphosis correction. The mean correction of local kyphosis at the osteotomy site was 30.9 degrees. Minor surgical complications, though present, were managed effectively.</p><p><strong>Conclusion: </strong>While acknowledging surgical complexities and potential complications, meticulous patient selection and appropriate techniques mitigate risks. This study emphasizes the pivotal role of surgical intervention in improving quality of life, particularly by addressing sagittal vertical axis and forward gaze disorders.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"11"},"PeriodicalIF":2.1,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11771011/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143051030","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 rare intersection: squamous cell carcinoma of the tonsil and the anti-TIF1 syndrome masquerade.","authors":"Manush Sondhi, Megan Lear, Saleha Dar, Madiha Tariq","doi":"10.1186/s41927-025-00460-8","DOIUrl":"10.1186/s41927-025-00460-8","url":null,"abstract":"<p><strong>Background: </strong>Dermatomyositis is a chronic inflammatory condition affecting muscles and skin, often associated with an increased risk of cancer. Specific autoantibodies, including anti-TIF1 (Transcription Intermediary Factor 1), have been linked to this risk. We present a case of dermatomyositis in a male patient positive for anti-TIF1 antibodies, subsequently diagnosed with squamous cell carcinoma of the tonsil, a novel association not previously documented. Early recognition of such associations is crucial for timely intervention and improved outcomes in these patients.</p><p><strong>Case presentation: </strong>A 53-year-old Caucasian male with hyperlipidemia presented with chronic dry, scaly skin and pruritus, diagnosed with eczematous dermatitis. Despite treatment, symptoms persisted. After two years, he reported increased redness of the rash and new eruptions on his hands and fingers. During a rheumatology visit, he reported weight loss, fatigue, muscle weakness, and trismus. Further evaluation indicated signs of dermatomyositis, and laboratory tests revealed anti-TIF1 antibodies, prompting further investigation. The patient underwent age-appropriate cancer screening, and due to a known association with malignancy, a positron emission scan was ordered, detecting increased activity in the right tonsil. Subsequent magnetic resonance imaging showed a suspicious mass in the tonsillar area. A biopsy confirmed invasive squamous cell carcinoma positive for P16+. Initial treatment included radiotherapy, with a post-treatment PET scan showing no evidence of disease. However, four months later, the cancer recurred, leading to significant symptoms and complications. Despite supportive measures, the patient succumbed to high-volume oral cavity bleeding during hospitalization.</p><p><strong>Conclusions: </strong>TIF1 dermatomyositis is a unique subset of dermatomyositis with a strong association with malignancy, particularly squamous cell carcinoma (SCC). Mechanisms connecting TIF1 dermatomyositis and cancer involve gene expression dysregulation and chronic inflammation. Anti-TIF1 antibodies are key biomarkers, with IgG2 isotype levels highly predictive of cancer risk. Common malignancies include ovarian, breast, and lung cancers, often detected within three years of dermatomyositis onset. Distinctive features include severe skin lesions, dysphagia, and minimal interstitial lung disease. Management focuses on early cancer detection and treatment, with options for refractory disease, including IVIg, rituximab, and emerging therapies like JAK inhibitors. Our case highlights a new association between TIF1α antibodies and tonsil squamous cell carcinoma. Despite successful radiotherapy, cancer recurred. TIF1 antibody detection should prompt rigorous cancer screening, emphasizing multidisciplinary management.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"9"},"PeriodicalIF":2.1,"publicationDate":"2025-01-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11765931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143045678","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}