Clinical and experimental rheumatology最新文献

{"title":"IL-1 targeting agents in Schnitzler syndrome: a multicentre, real-world study from the international AIDA Network Schnitzler Registry.","authors":"Laura Calabrese, Alessandra Cartocci, Antonio Vitale, Eduardo Martín-Nares, Martina D'Onghia, Valeria Caggiano, Jiram Torres-Ruiz, Roberta Lopez, Karina Jahnz-Rozyk, Katarzyna Rybak, Micol Frassi, Franco Franceschini, Francesca Crisafulli, Abdurrahman Tufan, Hamit Kucuk, Aslihan Avanoglu Guler, Antonio Gidaro, Leyla La Cava, Francesca Della Casa, Ilaria Mormile, Elisa Cinotti, Giuseppe Lopalco, Jurgen Sota, Jessica Sbalchiero, Giacomo Emmi, Andreas Recke, Stefania Costi, Paolo Sfriso, Sara Monti, Ombretta Viapiana, Andrea Hinojosa-Azaola, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Pietro Rubegni, Ewa Wiesik-Szewczyk, Luca Cantarini","doi":"10.55563/clinexprheumatol/i9hsfr","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/i9hsfr","url":null,"abstract":"<p><strong>Objectives: </strong>Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterised by a primary pathogenic involvement of interleukin (IL)-1. Therefore, IL-1 blockers are currently considered the optimal therapeutic option for SchS patients. However, while IL-1 blockers are first-line for SchS, long-term real-world evidence is limited by the rarity of the disease. We assessed the long-term effectiveness and safety of the IL-1 inhibitors anakinra and canakinumab used in SchS, also looking for variables capable of affecting global effectiveness and drug retention over time.</p><p><strong>Methods: </strong>Data analysed in this study were drawn from the international AutoInflammatory Disease Alliance (AIDA) Registry dedicated to SchS.</p><p><strong>Results: </strong>28 SchS patients corresponding to 37 treatment lines were included in the study. Complete and partial responses occurred in 73.1% and 29.9% of anakinra-treated patients, and 66.8% and 33.3% with canakinumab. The overall anakinra and canakinumab drug retention rates at 12-, 36-, and 60-month follow-up were 85.6%, 81.7% and 64.7%, respectively; the probability of discontinuing IL-1 inhibitors at 12-, 36- and 60 months due to loss of effectiveness was 9.6%, 13.7% and 24.5%, respectively. The maximum IgG M-protein levels were found to be significantly higher in patients achieving partial response compared to those benefiting from complete response (p=0.032). Lymphadenopathy independently predicted anti-IL-1 discontinuation due to loss of effectiveness (HR 7.78, 95% CI: 1.27-47.9; p=0.027).</p><p><strong>Conclusions: </strong>The present study confirms the high effectiveness of IL-1 inhibitors in controlling SchS, including the complete and partial response rates and the long-term survival. Elevated IgG M-protein levels and the presence of lymphadenopathy should be considered as potential indicators for identifying patients more likely to exhibit a partial response and a possible loss of treatment efficacy.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 10","pages":"1753-1762"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145353663","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of post-operative cardiovascular complications for Behçet's disease with pre-operative immunosuppressive therapy.","authors":"Lanxin Ye, Fanyu Chen, Weiteng Wang, Oudi Chen, Hongkun Qing, Lixi Gan, Lequan Zhuang, Yang Cui, Xuhua Jian","doi":"10.55563/clinexprheumatol/gkx830","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/gkx830","url":null,"abstract":"<p><strong>Objectives: </strong>Patients with Behçet's disease (BD) with cardiovascular involvement often have more post-operative complications in correcting the pathology by surgical means. This study aims to explore the benefits of pre-operative immunosuppressive therapy, predict complications using inflammatory biomarkers, and evaluate optimal surgery timing.</p><p><strong>Methods: </strong>This retrospective study analysed predictors of post-operative complications in BD patients who underwent cardiovascular surgery with perioperative immunosuppressive therapy at Guangdong Provincial People's Hospital from 2012 to 2024.</p><p><strong>Results: </strong>In-hospital complications were lower in patients who received pre-operative immunosuppressive therapy (9% vs. 58.8%, p<0.001). Rheumatoid factor (RF, hazard ratio [HR] 1.088; 95% confidence interval [CI], 0.998-1.187; p=0.056), platelet-to-lymphocyte ratio (PLR, HR 1.004; 95% CI, 1.000-1.008; p=0.075), and neutrophil-to-lymphocyte ratio (NLR, HR 1.065; 95% CI, 1.002-1.133; p=0.045) were identified as independent risk factors for post-operative complications, while pre-operative immunosuppressive therapy (HR 0.206; 95% CI, 0.061-0.693; p=0.011) was a protective factor. The area under the curve (AUC) for the receiver operating characteristic curve for four or more positive biomarkers was 0.849.</p><p><strong>Conclusions: </strong>Pre-operative immunosuppressive therapy is vital for BD patients. Monitoring inflammatory biomarkers helps identify the best timing for surgery and reduces complications.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 10","pages":"1763-1771"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145353722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinicopathological characteristics of severe aortic valve regurgitation caused by Behçet's syndrome.","authors":"Menghao Zhang, Xun Wang, Yeling Liu, Xinpei Liu, Xin Yu, Luxi Sun, Zhimian Wang, Lifan Zhang, Jinjing Liu, Guotao Ma, Wei Chen, Wenze Wang, Qi Miao, Wenjie Zheng","doi":"10.55563/clinexprheumatol/k2v1he","DOIUrl":"10.55563/clinexprheumatol/k2v1he","url":null,"abstract":"<p><strong>Objectives: </strong>Aortic valve regurgitation (AR) caused by Behçet's syndrome (BS) has high mortality. Preoperative biologics reduced systemic inflammation, but their effect on lesion inflammation remains unclear.</p><p><strong>Methods: </strong>Twenty-two BS patients with severe AR who underwent cardiac surgery with retained pathological specimens were included. The pathology of the aortic wall and/or valve was re-analysed based on their preoperative disease activity and treatment strategy. Immunohistochemistry (IHC) assessed the distribution of CD4+, CD8+, CD20+ and CD68+ cells.</p><p><strong>Results: </strong>The mean diagnosis age was 39.6±13.1 years, with a median disease duration of 9 (3-35) years. Seven (31.8%) underwent cardiac surgery during the active phase due to uncontrollable disease progression, while 15 (68.2%) were in remission. Pathologically, severe AR caused by BS is characterised by mixed inflammatory cell infiltration in the aortic wall. Active cases showed significantly more diffuse infiltration of CD4+ (100% vs. 8.3%, p=0.0002) and CD8+ (71.4% vs. 20%, p=0.058) T cells in the aortic adventitia, with more neutrophil infiltration in the aortic valve (60% vs. 7.7%, p=0.044). Notably, less CD68+ macrophage infiltration (57.2% vs. 0%, p=0.045), CD4+ T cell diffusion (57.1% vs. 0%, p=0.045), and vasa vasorum mucoid degeneration (85.7% vs. 20%, p=0.017) were observed in the aortic adventitia of patients receiving preoperative biologics, together with less aortic valve necrosis (71.4% vs. 0%, p=0.023).</p><p><strong>Conclusions: </strong>Overall, our study provides valuable insights into the pathology of severe AR caused by BS as a mixed inflammatory infiltration and provides the first pathological rationale for achieving preoperative remission and early biologics to improve the prognosis.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1726-1734"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143779039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The relationship between NETosis findings and disease activity in Behçet's disease: an exploratory study.","authors":"Erdem Bektas, Rabia Deniz, Zeliha Emrence, Sema Sirma Ekmekci, Neslihan Abaci, Shirkhan Amikishiyev, Yasemin Yalcinkaya, Bahar Artim Esen, Murat Inanc, Ahmet Gül","doi":"10.55563/clinexprheumatol/4thkqz","DOIUrl":"10.55563/clinexprheumatol/4thkqz","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to investigate the relationship between the neutrophil extracellular traps (NET) and NETosis findings and disease activity in Behçet disease (BD).</p><p><strong>Methods: </strong>The study group consisted of 30 active BD patients and 10 healthy individuals as controls. Serum and saliva samples were collected from the patients during their active and remission periods. A subset of them were followed longitudinally. Serum and saliva cf-DNA, NE, MPO, and cit-H3 levels were measured as indirect NETosis findings, subsequently the results were adjusted based on the peripheral blood neutrophil counts. Unadjusted and adjusted levels of the NETosis findings were evaluated.</p><p><strong>Results: </strong>In active BD, unadjusted serum cf-DNA and NE levels were significantly higher than in controls, whereas adjusted serum MPO and cit-H3 levels were found to be lower. In inactive BD, unadjusted serum NE levels remained elevated compared to controls, while unadjusted serum MPO, adjusted serum MPO, and adjusted serum cit-H3 levels were lower. No significant differences were observed in salivary NETosis findings between the patients and controls. Longitudinal follow-up revealed a decrease in both unadjusted serum cf-DNA and saliva cf-DNA levels in parallel with reduced clinical activity. Saliva and unadjusted serum cf-DNA showed a positive correlation with inflammatory markers, whereas adjusted serum MPO and cit-H3 correlated negatively.</p><p><strong>Conclusions: </strong>Indirect NETosis findings varied in relation to the systemic and/or local activity of BD patients. The changes after adjustment suggest that serum NETosis markers can be influenced by increased neutrophil turnover during the active phase of the disease.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1772-1781"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145174006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence, incidence and geographic distribution of familial Mediterranean fever in Turkey: a national cohort study.","authors":"Hasan Satiş, Ahmet Gül, Gizem Ayan, Hakan Babaoğlu, Berkan Armağan, Abdulsamet Erden, Emre Bilgin, Erdem Karabulut, Levent Kiliç, Duygu Tecer, Alper Sari, Orhan Küçükşahin, Serdal Uğurlu, Özgür Kasapçopur, Seza Özen, Mustafa Okan Ayvali, Ali Aykut Fidanci, Mustafa Mahir Ülgü, Naim Ata, Şuayip Birinci, Umut Kalyoncu","doi":"10.55563/clinexprheumatol/pzbg88","DOIUrl":"10.55563/clinexprheumatol/pzbg88","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to analyse the incidence and geographical distribution of Familial Mediterranean Fever (FMF) in Turkey using the electronic medical records database (e-Pulse) of the Ministry of Health.</p><p><strong>Methods: </strong>The study utilised nationwide health data from the e-Pulse, which has been operational since 2016. Patient selection was based on ICD-10 codes for FMF, with a minimum of two recorded codes entered at least 30 days apart. Patients aged ≥50 and those with gout-related ICD-10 codes were excluded. The prevalence and incidence of FMF in 2018 were calculated, taking into account gender, age demographics, and regional distribution.</p><p><strong>Results: </strong>A total of 160,897 FMF patients were identified from a population of 82,003,882, yielding a prevalence of 139 per 10,000 individuals. The incidence was 2.78 per 10,000. The highest number of records was found among individuals aged 15-19. Geographically, the highest rate of prevalence was found in Ardahan, Bayburt, and Sivas, regions in the North-Eastern part of Turkey. Family records revealed that 11.7% of children under 18 with FMF had at least one parent diagnosed with FMF.</p><p><strong>Conclusions: </strong>FMF is beyond the definition of a rare disease and a significant health issue in Turkey, with a non-uniform distribution influenced by both genetic and historical factors. The findings of this study highlight the utility of national electronic health records like e-Pulse in conducting large-scale epidemiological research, which could guide future public health strategies for FMF patients.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1709-1714"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145198279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Identification of clinical phenotypes in Behçet's syndrome using latent class analysis: a step toward precision medicine.","authors":"Sarra Chadli, Mouna Maamar, Redouane Abouqal, Wafaa Ammouri, Zoubida Tazi Mezalek, Hicham Harmouche","doi":"10.55563/clinexprheumatol/3dhh59","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/3dhh59","url":null,"abstract":"<p><strong>Objectives: </strong>Behçet's syndrome (BS) is characterised by extreme clinical heterogeneity, underscoring the need for precise patient classification to enable personalised management. While traditional distance-based cluster analysis (CA) has provided new insights, its deterministic approach may not fully capture the complexity of BS. The primary objective of this study was to define BS clinical phenotypes using latent class analysis (LCA), a probabilistic, model-based clustering method that identifies hidden classes based on unobserved patterns. We also aimed to examine sex-related differences in clinical manifestations and treatment requirements across the identified classes.</p><p><strong>Methods: </strong>We conducted a retrospective, observational, single-centre study including all adult BS patients followed in our department between 2012 and 2022, targeting a sample of 500 patients. LCA was performed using clinically relevant indicators (sex, oral and genital ulcers, skin lesions, articular involvement and major organ involvement). Models were compared based on fit indices, class number, separation, assignment and size. The final model was selected based on both clinical relevance and statistical performance.</p><p><strong>Results: </strong>A total of 553 patients (409 males, 144 females) were enrolled, with a mean age of 32±7 years. Five latent classes (C1-C5) with distinct phenotypes were identified. C1 (n=215; 39%), 'vascular type': all patients had vascular lesions, with the highest prevalence of cardiac involvement (12%). C2 (n=171; 31%), 'ocular type': characterised by 100% uveitis and frequent mucocutaneous lesions. C3 (n=40; 7%), 'neurological type': all patients exhibited parenchymal neurological involvement, and 40% had concomitant uveitis. C4 (n=98; 18%), 'skin-mucosa and articular type': marked by 100% oral and genital ulcers, with the highest prevalence of papulopustular lesions (54%) and articular involvement (48%). C5 (n=29; 5%), 'uncertain BS': with 60% uveitis, 48% vascular lesions, and the lowest mucocutaneous involvement. Sex-related clinical differences were observed, with significant male predominance across all major organ classes (C1, C2, C3, and C5), whereas a near-equal sex distribution was noted in the skin-mucosa and articular class (p<0.001). Treatment patterns varied considerably, with higher corticosteroid doses and conventional immunosuppressant use in major organ classes, while biologics were mostly prescribed in the 'ocular class' (C2) and 'uncertain BS' (C5) (p< 0.001).</p><p><strong>Conclusions: </strong>This study is the first to apply LCA for BS clinical phenotyping, providing a probabilistic classification that uncovers complex patient subgroups. Five latent classes were identified, with distinct clinical profiles, significant sex disparities, and varying therapeutic needs. These findings are crucial for advancing precision medicine in BS and ultimately improving patient outcomes.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 10","pages":"1789-1798"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145353642","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Promoting patients' empowerment in rheumatic diseases: the perspective of the International Society of Behçet's Disease Working Group on Patient Empowerment, Adherence to Therapy and Patients' Education.","authors":"Federica Di Cianni, Diana Marinello, Andreas Altenburg, Umit Karacayli, Giacomo Emmi, Michael Schirmer, Gonca Mumcu, Farida Fortune, Rosaria Talarico","doi":"10.55563/clinexprheumatol/rk42aa","DOIUrl":"10.55563/clinexprheumatol/rk42aa","url":null,"abstract":"<p><p>Behçet's disease (BD) is a rare multisystemic vasculitis that significantly impacts patients' quality of life. Effective management of BD requires a patient-centred approach that empowers individuals to actively participate in their care. This work explores the importance of patient empowerment, adherence to treatment, and patient education in BD. The impact of BD on quality of life (QOL) is significant, affecting both physical and psychological well-being. QOL measures are essential in capturing the full burden of the disease from the patient's perspective, helping guide interventions that can improve disease management. Moreover, treatment adherence remains a major challenge due to various factors, including medication complexity, fear of side effects, and patient perceptions of their health status. Empowerment strategies, including education and emotional support, are crucial to improving adherence and reducing healthcare costs. Empowering BD patients involves fostering a collaborative relationship between healthcare providers and patients, promoting shared decision-making and enhancing patients' knowledge and skills to manage their condition. Patient education plays a crucial role in empowering patients by providing them with accurate information about the disease, treatment options, and self-management strategies. The International Society for Behçet's Disease Working Group on Patient Empowerment, Adherence to Therapy, and Patient Education aims to address these issues by developing global strategies to empower BD patients, caregivers and healthcare providers. This initiative promotes collaboration across the BD community, ultimately leading to better patient outcomes and serving as a model for similar efforts in other disease areas.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1810-1815"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145029025","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Behçet's disease beyond the criteria: insights from a tertiary centre in Turkey.","authors":"Busra Firlatan Yazgan, Ezgi Aysu Sahin Mavi, Gozde Sevgi Kart Bayram, Bugu Bulat, Mustafa Ekici, Erdinc Unaldi, Gul Sandal Uzun, Gizem Ayan, Zehra Ozsoy, Serife Asya Germe, Bayram Farisogullari, Emine Duran, Gozde Kubra Yardimci, Ertugrul Cagri Bolek, Emre Bilgin, Berkan Armagan, Alper Sari, Abdulsamet Erden, Levent Kilic, Bunyamin Kisacik, Yuksel Maras, Ismail Dogan, Omer Karadag, Ali Akdogan, Sule Apras Bilgen, Sedat Kiraz, Ali Ihsan Ertenli, Umut Kalyoncu","doi":"10.55563/clinexprheumatol/h8mxc6","DOIUrl":"10.55563/clinexprheumatol/h8mxc6","url":null,"abstract":"<p><strong>Objectives: </strong>There are no pathognomonic findings for diagnosing Behçet's disease (BD), the diagnosis relying primarily on clinical evaluation. We aimed to assess patients with BD based on two classification criteria the International Study Group (ISG) criteria and the International Criteria for Behçet's Disease (ICBD), both at diagnosis and throughout the disease, and to explore the characteristics of patients who do not meet the criteria but are clinically diagnosed with BD.</p><p><strong>Methods: </strong>Patients with BD were identified from the Hacettepe University Vasculitis Research Centre database. Paediatric cases and those with incomplete clinical data were not included. Demographics, clinical characteristics, and treatments were assessed. The physician's clinical judgment determined the gold standard for diagnosis. The patients' fulfilment of the ISG and ICBD criteria was evaluated at diagnosis and during the follow-up.</p><p><strong>Results: </strong>The study included 804 patients with BD (49.8% males). The mean age at diagnosis was 28.9±10.4 years. At diagnosis, 52.6% of patients fulfilled the ISG criteria, and 81.0% met the ICBD criteria. During follow-up, these rates increased to 59.0% and 84.7%, respectively. Significant organ involvement (ocular, vascular, or neurological) was present in 47.9% of patients not meeting ISG criteria and 30.9% of those not meeting ICBD criteria. The use of at least one immunosuppressive agent was 48.5% and 38.2% in these groups, respectively.</p><p><strong>Conclusions: </strong>According to various classification criteria, 15-40% of patients with BD do not meet established classification criteria at any point during follow-up, yet significant organ involvement remains prevalent. These findings highlight the limitations of strict criteria-based diagnosis and underscore the importance of clinical expertise in recognising and managing BD.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1782-1788"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145257552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inflammatory arthritis as an atypical manifestation of Coffin-Siris syndrome linked to a novel ARID1B variant.","authors":"Sigal Matza-Porges, Zahala Bar-On, Atar Lev, Amos J Simon, Hagit Peleg, Oded Shamriz","doi":"10.55563/clinexprheumatol/833pi0","DOIUrl":"10.55563/clinexprheumatol/833pi0","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1838-1839"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144224535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"On-demand corticosteroid use in the syndrome of undifferentiated recurrent fever: a literature review and results from the JIR-CLiPS survey study.","authors":"Ezgi Deniz Batu, Seher Sener, Mariana Rodrigues, Caroline Vinit, François Hofer, Katerina Laskari, Ricardo Craveiro Costa, Margarida Santos Faria, Gulcan Ozomay Baykal, Oksana Boyarchuk, Olivier Gilliaux, Konstantinos Pateras, Hafize Emine Sonmez, Natasa Toplak, Marco Gattorno, Michaël Hofer","doi":"10.55563/clinexprheumatol/jmtag4","DOIUrl":"10.55563/clinexprheumatol/jmtag4","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to analyse the strategies of physicians regarding corticosteroid use in syndrome of undifferentiated recurrent fever (SURF) and examine the published data on this topic.</p><p><strong>Methods: </strong>The JIR-CliPS questionnaire which addresses physicians' practices about on-demand corticosteroid use in SURF was distributed via e-mail to potential respondents. We systematically reviewed the MEDLINE and Scopus databases and extracted the data about on-demand corticosteroid use in SURF.</p><p><strong>Results: </strong>One hundred thirty-seven physicians (F/M=2.5; 66.4% paediatric rheumatologists) from 45 countries responded to the survey. Around 70% of physicians prescribe corticosteroids for SURF flares. Most physicians (81.9%) do not use corticosteroids in SURF patients routinely, and this practice is more common among less experienced physicians (p<0.001). Prednisolone at a dose of 1 mg/kg (54.4%) was the most commonly preferred corticosteroid. The most common definition of response to corticosteroids was 'response within 12 hours' (51.6%). Most respondents (59.5%) consider changing treatment if corticosteroids cause a decrease in quality of life. We found 10 articles in the literature describing 239 SURF patients treated with on-demand corticosteroids. The most frequently preferred corticosteroid was prednisolone (63.8%). The response to corticosteroids was 70.8% and an increase in attack frequency was observed in almost 40% of patients.</p><p><strong>Concusions: </strong>On-demand corticosteroid use is not uncommon in the acute management of SURF attacks. However, most physicians do not use corticosteroids routinely and there is no consensus regarding the definition of response to treatment and when to change treatment neither in our survey results nor in the literature.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"1816-1822"},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144076356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}