Clinical and experimental rheumatology最新文献

{"title":"ABO blood groups and increased risk for the development of vascular involvement in Behçet's disease.","authors":"Erdem Bektas, Abdülkadir Büyükdemir, Yasemin Yalcinkaya, Bahar Artim Esen, Murat Inanc, Ahmet Gül","doi":"10.55563/clinexprheumatol/475an5","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/475an5","url":null,"abstract":"<p><strong>Objectives: </strong>Inflammatory endothelial activation is a prominent feature of vascular involvement in Behçet's disease (BD), which is usually accompanied by a thrombotic tendency. Recent studies suggest an association between ABO blood groups and thrombotic vascular disease in those carrying non-O (A, B, and AB) groups. This study aimed to analyse the contribution of ABO blood groups to the vascular involvement in BD.</p><p><strong>Methods: </strong>In this retrospective study, BD patients with available ABO blood group data and meeting the ISG criteria were included in the study. The presence of vascular involvement, its clinical characteristics, and the data about other manifestations were recorded from the patient charts.</p><p><strong>Results: </strong>This study was conducted in 411 patients; 143 (34.8%) were carrying O and 268 (65.2%) were carrying non-O groups. The vascular involvement was significantly more frequent and the risk for overall vascular and venous events was up to 2-fold higher in the patients with non-O groups. After adjustments for age, sex, and comorbidities, the risk for arterial disease was also found to be increased in association with non-O groups. ABO blood groups were shown to be independent risk factors for vascular BD by multiple regression models based on known predisposing factors. Compared with other non-O blood groups, the patients carrying blood group B had a higher risk for vascular events.</p><p><strong>Conclusions: </strong>The results of this preliminary study show the potential contribution of ABO blood groups to the vascular-BD phenotype and suggest an increased risk for vascular BD in association with non-O blood groups.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"42 10","pages":"2071-2075"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459412","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":"Reply to: Addressing key missing variables in reproductive health studies in familial Mediterranean fever.","authors":"Pavel Sotskiy, Olga Sotskaya, Tamara Sarkisian, Hasmik Hayrapetyan, Marina Safaryan, Eldad Ben-Chetrit","doi":"10.55563/clinexprheumatol/340p9k","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/340p9k","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"42 10","pages":"2104"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459418","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":"Infliximab for parenchymal neuro-Behçet's syndrome: case series and meta-analysis.","authors":"Xiaoou Wang, Yeling Liu, Yiyuan Ao, Jing Wang, Jinjing Liu, Yan Xu, Wenjie Zheng","doi":"10.55563/clinexprheumatol/5lnkd4","DOIUrl":"10.55563/clinexprheumatol/5lnkd4","url":null,"abstract":"<p><strong>Objectives: </strong>This study aims to evaluate the efficacy and safety of infliximab (IFX) in patients with parenchymal neuro-Behçet's syndrome (p-NBS).</p><p><strong>Methods: </strong>We retrospectively analysed eleven p-NBS patients treated with IFX at our institution and combined them with studies from database searches for a meta-analysis. Pooled estimates of clinical response (complete and partial remission) and MRI improvement at months 3, 6, and 12 were calculated.</p><p><strong>Results: </strong>One patient achieved CR and the other ten patients achieved PR at our institution. 8 studies (77 patients) were included in the meta-analysis. At 3, 6, and 12 months, 97% (95%CI 61.9-100%), 89.6% (95%CI 45.9-100%), 100% (95%CI 96.0-100%) of patients showed clinical response and 100% (95%CI 89.7-100%), 89.1% (95% CI 26.3-100%), 99.5% (95% CI 96.0-100%) of patients showed radiological improvement, respectively. Severe adverse events were observed in 7 patients.</p><p><strong>Conclusions: </strong>IFX was effective and relatively safe for p-NBS. Patients should be re-evaluated after 3 months of IFX to determine further therapy.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"2040-2048"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140956582","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":"Reproductive disorders in homozygote and heterozygote patients with familial Mediterranean fever patients and controls.","authors":"Pavel Sotskiy, Olga Sotskaya, Tamara Sarkisian, Hasmik Hayrapetyan, Eldad Ben-Chetrit, Marina Safaryan","doi":"10.55563/clinexprheumatol/9yc77f","DOIUrl":"10.55563/clinexprheumatol/9yc77f","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"2102"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364612","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":"2nd Familial Mediterranean Fever Meeting.","authors":"Huri Özdoğan, Eldad Ben-Chetrit, Serdal Uğurlu","doi":"10.55563/clinexprheumatol/9o6qg4","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/9o6qg4","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"42 10","pages":"2107-2130"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459411","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 syndrome: one year in review 2024.","authors":"Gülen Hatemi, Emire Seyahi, Izzet Fresko, Rosaria Talarico, Didar Uçar, Vedat Hamuryudan","doi":"10.55563/clinexprheumatol/fqao16","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/fqao16","url":null,"abstract":"<p><p>The aim of this review is to provide a critical summary of studies published during 2023 that contribute to our understanding of Behçet's syndrome. An increase in the incidence of BS was reported in Northern Spain after 2014.Studies on patient perspectives showed the impact of Behçet's syndrome on quality of life, daily activities, education, work, and relationships. Differences in genetics among Behçet's syndrome patients with different types of organ involvement were reported and an association between HLA-B/MICA and SLCO4A1 polymorphisms and eye involvement and between DDX60L polymorphisms and nervous system involvement were observed. It was suggested that Integrin α9β1 plays a crucial role in the neutrophil-mediated inflammatory pathways underlying this syndrome and the dysfunction of the PDL1/PD-1 pathway may be associated with the hyperactivity of the Th-1/Th-17 axis. Vein wall thickness seems to be increased in patients with Behçet's syndrome, but its pathogenetic and clinical implications are not clear. There is growing evidence regarding the efficacy of TNF inhibitors in different types of organ involvement. A number of small case series point out to the potential role of TCZ and JAK inhibitors.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"42 10","pages":"1999-2007"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459414","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":"Markedly elevated serum IL-6 levels predict relapse within six months of treatment initiation in Still's disease.","authors":"Hideki Oka, Shuji Sumitomo, Hayato Shimizu, Koichiro Ohmura","doi":"10.55563/clinexprheumatol/611dkl","DOIUrl":"10.55563/clinexprheumatol/611dkl","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"2100-2101"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142124999","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":"Atrial myxoma as a mimicker of intracardiac thrombus in Behçet's syndrome: a case study with histopathological confirmation.","authors":"Tumay Ak, Bahar Bayrakdar, Sebnem Batur, Emine Sebnem Durmaz, Murat Cimci, Emire Seyahi","doi":"10.55563/clinexprheumatol/j29rnr","DOIUrl":"10.55563/clinexprheumatol/j29rnr","url":null,"abstract":"<p><p>Cardiomyopathies cause most intracardiac thrombosis (ICT), and Behçet's syndrome (BS) is a rare inflammatory disease that can be responsible for a proportion of ICT. Other inflammatory disorders involved in the aetiology of ICT include antiphospholipid syndrome, Henoch-Schonlein purpura, COVID-19, and Loeffler endocarditis. ICT usually occur during the active phase of BS, and they have a close relationship with vascular involvement. Atrial myxomas are benign cardiac tumours arising from the interatrial septum. They can lead to a substantial acute phase response, making them difficult to distinguish from inflammatory diseases. In this case study, we present a 46-year-old female BS patient who presented with constitutional symptoms mimicking BS flare in a routine follow-up visit and was diagnosed with left atrial myxoma after administration of several lines of immunosuppressives. Then, she underwent surgical tumour excision, and a histopathological examination confirmed the diagnosis.In conclusion, atrial myxoma should be kept in mind first of all when suspecting ICT, and advanced imaging methods such as cardiac magnetic resonance imaging (MRI) should be used if necessary.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"2092-2095"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141615968","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":"Autoinflammatory syndromes mimicking Behçet's disease with gastrointestinal involvement: a retrospective analysis.","authors":"Qianying Lv, Yifan Li, Qijiao Wei, Tianchao Xiang, Wanzhen Guan, Yinv Gong, Qiaoqian Zeng, Xiaomei Zhang, Yuhuan Wang, Yu Shi, Haimei Liu, Hong Xu, Li Sun","doi":"10.55563/clinexprheumatol/g6729b","DOIUrl":"10.55563/clinexprheumatol/g6729b","url":null,"abstract":"<p><strong>Objectives: </strong>This retrospective study aimed to investigate the clinical characteristics and genetic findings in paediatric patients with gastrointestinal involvement in Behçet's disease (BD), elucidating the spectrum of autoinflammatory syndromes mimicking BD in this young population.</p><p><strong>Methods: </strong>Fifty paediatric patients diagnosed with BD between January 2016 and December 2022, including 24 (48%) with gastrointestinal involvement, underwent comprehensive analysis. Clinical presentations, laboratory examinations, gastrointestinal endoscopy, and genetic tests were conducted, with patients stratified based on genetic results for rigorous comparative clinical analysis.</p><p><strong>Results: </strong>The cohort, with a median age of disease onset at 4.0 years, predominantly manifested with recurrent oral ulcers (100%). Gastrointestinal symptoms were prevalent in 83.3%, with abdominal pain (70%) and haematochezia (16.7%) being notable. Endoscopic evaluations unveiled lesions primarily in the terminal ileum and ileocecal region, with diverse ulcers across various anatomical sites. While 70.8% initially met ICBD criteria, only 41.6% fulfilled new paediatric classification criteria. Genetic analysis in 18 patients unveiled pathogenic variants in 7, with the genetic-positive group exhibiting earlier onset and more atypical symptoms. Noteworthy cases included X-linked deficiency in ELF4, A20 haploinsufficiency, and Majeed syndrome, with two cases revealing chromosomal abnormalities such as trisomy 8 syndrome. Comparative analysis underscored earlier disease onset, heightened inflammatory markers, and distinctive gastrointestinal lesions in the genetic-positive cohort.</p><p><strong>Conclusions: </strong>Identification of monogenic diseases and chromosomal abnormalities resembling BD underscores the imperative of precise diagnosis for tailored treatment and genetic counselling. Expanding genetic screening initiatives holds promise for enhancing our comprehension of the genetic landscape associated with BD.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"2076-2085"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364633","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":"In Memoriam of Masaki Takeuchi.","authors":"Daniel L Kastner, Akira Meguro, Ahmet Gül","doi":"10.55563/clinexprheumatol/db8ryv","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/db8ryv","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"42 10","pages":"0"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459416","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}