Clinical and experimental rheumatology最新文献

{"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":"https://doi.org/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":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-02","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":"Primary versus systemic-sclerosis-associated Raynaud's phenomenon: relationship with clinical and environmental factors.","authors":"Elvira Favoino, Marcella Prete, Vasiliki Liakouli, Adriana Sisto, Ada Corrado, Patrizia Leone, Giuseppe Lisco, Marta Vomero, Edoardo Biancalana, Emanuele Chiara, Giacomo Emmi, Vito Racanelli, Antonella Marcoccia, Rosa Daniela Grembiale, Francesco Paolo Cantatore, Luca Navarini, Piero Ruscitti, Francesco Ciccia, Roberto Giacomelli, Federico Perosa","doi":"10.55563/clinexprheumatol/m7qpn3","DOIUrl":"10.55563/clinexprheumatol/m7qpn3","url":null,"abstract":"<p><strong>Objectives: </strong>Raynaud's phenomenon (RP) can be induced by stress and environmental factors, occurring as a primary disease (pRP) or associated with connective tissue disease. RP is seen in more than 95% of patients with systemic sclerosis (SSc) and may precede its diagnosis by several years. Accordingly, there is a clear need to identify those patients with RP who will eventually develop connective tissue disease, including SSc. The aim of this case-control study was to assess the association of SSc-RP versus pRP with respect to environmental factors, lifestyle habits, and clinical setting.</p><p><strong>Methods: </strong>A questionnaire was used to collect current data from 180 patients with SSc-RP and 103 with pRP. Statistical analyses were performed to identify possible risk factors for SSc-RP.</p><p><strong>Results: </strong>SSc-RP was found to be inversely associated with living in urban area (OR=0.37; p<0.001), computer use (OR=0.38, p<0.001), contraceptive use (OR=0.32; p=0.017), habitual alcohol use (OR=0.35; p=0.029), and hepatitis B virus vaccine (OR=0.09; p=0.011),while it was directly associated to cold sensitivity (OR=3.48; p=0.001), lower quality of life (OR=2.69; p<0.001), finger pain (OR=3.03; p<0.001) and autoimmune hypothyroidism (OR=3.62; p=0.007). All associations were supported by either multivariate and/or multivariable analyses.</p><p><strong>Conclusions: </strong>This study revealed differences in lifestyle and preventive health behaviours between SSc-RP and pRP, and also suggests that patients with pRP and autoimmune hypothyroidism should be strictly monitored for any clinical changes that may indicate SSc onset. Further investigations are needed to prospectively evaluate autoimmune hypothyroidism as a predisposing condition for SSc-RP.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143779101","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":"Causal relationship between 731 immune cell immunophenotypes and giant cell arteritis: a Mendelian randomisation study.","authors":"Qiong Liu, Xiaofang Liu, Mengge Gao, Bo Yang, Miaoqing Luo, Biying Yang, Guojun Liang","doi":"10.55563/clinexprheumatol/bnjlz9","DOIUrl":"10.55563/clinexprheumatol/bnjlz9","url":null,"abstract":"<p><strong>Objectives: </strong>Giant cell arteritis (GCA) is the most common form of vasculitis among adults aged 50 and over, characterised by systemic inflammation and the potential for severe complications such as blindness and stroke. Despite its prevalence, the aetiology of GCA remains incompletely understood, with current treatments largely relying on corticosteroids, which carry significant side effects.</p><p><strong>Methods: </strong>Our study utilised a bilateral Mendelian randomisation (MR) approach to investigate the causal impact of immune cells on GCA. By analysing 731 immune cell phenotypes from genome-wide association studies (GWAS) data of 3,757 European individuals, we aimed to identify genetic variants as instrumental variables for immune cell traits, thereby elucidating their role in GCA susceptibility. To ensure a robust examination, we used various MR techniques, including the inverse-variance weighted (IVW) method, and carried out sensitivity analyses to assess the dependability of our findings.</p><p><strong>Results: </strong>Forward MR analysis identified three immune traits with significant associations with GCA: a protective effect from the absolute count of monocytic myeloid-derived suppressor cells and increased risks associated with HLA DR expression on CD14+ CD16-, and CD14+ monocytes. The sensitivity analyses yielded results consistent with the main findings. The reverse MR analysis yielded no statistically significant results.</p><p><strong>Conclusions: </strong>The study advances our understanding of the immunological underpinnings of GCA, suggesting that specific immune cells significantly influence the disease's development. These insights pave the way for the exploration of new therapeutic targets that could offer more targeted and tolerable treatment options beyond the current reliance on corticosteroids. Further research is needed to validate these potential biomarkers and therapeutic targets in clinical settings.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"621-629"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735668","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":"Interleukin-6 inhibition for Takayasu's arteritis: before or after tumour necrosis factor inhibitors?","authors":"Tanaz A Kermani, Kenneth J Warrington","doi":"10.55563/clinexprheumatol/2acgxz","DOIUrl":"10.55563/clinexprheumatol/2acgxz","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"583-586"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735704","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":"Systemic vasculitis: one year in review 2025.","authors":"Paolo Delvino, Chiara Baldini, Martina Bonacini, Stefania Croci, Federica Di Cianni, Francesco Ferro, Chiara Marvisi, Sara Monti, Michele Moretti, Francesco Muratore, Ludovica Pisapia, Caterina Ricordi, Antonello Sulis, Rosaria Talarico, Elena Treppo, Luca Quartuccio, Carlo Salvarani","doi":"10.55563/clinexprheumatol/oyqz1p","DOIUrl":"10.55563/clinexprheumatol/oyqz1p","url":null,"abstract":"<p><p>Systemic vasculitides encompass a spectrum of inflammatory disorders affecting several organs and districts, with significant implications for morbidity and mortality. This annual review provides an updated overview of key advancements in vasculitis research, including emerging biomarkers, novel insights into pathogenesis, and therapeutic innovations in both large and small vessel vasculitis. Particular attention is given to emerging concepts, including the role of cellular senescence and stromal cells in vascular inflammation, the expanding spectrum of single-organ vasculitis, and the growing recognition of VEXAS syndrome as a vasculitis-related entity.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"553-562"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735713","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":"Sonographic study on vessel wall remodelling of the cranial and axillary arteries in giant cell arteritis under treatment: implications for diagnosis of relapses and impact of tocilizumab treatment.","authors":"Louise Füessl, Melike Findik-Kilinc, Lukas Caspar Thielmann, Christian Lottspeich, Ilaria Prearo, Christina Gebhardt, Hendrik Schulze-Koops, Michael Czihal","doi":"10.55563/clinexprheumatol/rnis5l","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/rnis5l","url":null,"abstract":"<p><strong>Objectives: </strong>Giant cell arteritis (GCA) is the most common primary systemic vasculitis and is nowadays commonly diagnosed using vascular ultrasound. Whether repeated ultrasound is helpful in disease management is unclear.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 100 patients diagnosed with GCA between 01/2016 and 12/2022. High-resolution ultrasound was performed to assess vasculitic wall thickening in superficial temporal, facial, and axillary arteries at diagnosis and during follow-up. Patients were treated according to current standards, with tocilizumab treatment initiated within 6 months after diagnosis in 38 patients. The course of wall thickening in the different vascular segments was recorded. Patients with and without complete normalisation of wall thickening were compared. The impact of tocilizumab treatment on vessel wall remodelling and the potential benefit of repeated ultrasound examinations for the diagnosis of relapsing disease were assessed.</p><p><strong>Results: </strong>In the overall cohort (63% females, mean age 72.8±8.9 years), one, two or three arterial territories were affected in 31, 50 and 17 patients. Follow-up ultrasound examinations showed a significant reduction in wall thickening over time: superficial temporal arteries -0.42 mm, facial arteries -0.35 mm, axillary arteries -0.36 mm. Normalisation of wall thickening occurred in 32.6% (superficial temporal arteries), 53.1% (facial arteries), and 35.5% (axillary arteries), with some differences in clinical characteristics between patients with and without complete sonographic remission. Patients treated with tocilizumab showed a slightly faster early reduction in mean intima-media thickness which was lost over time. Repeated ultrasound showed a significant increase in maximum IMT (at least +0.3 mm) in 3.6% of the superficial temporal arteries, 18.4% of the facial arteries, and 21.4% of the axillary arteries in patients with relapsing disease.</p><p><strong>Conclusions: </strong>Our results help to interpret repeated IMT measurements of the affected cranial and extracranial arteries in patients with GCA undergoing treatment. Repeated ultrasound examinations appear to be of limited diagnostic value in the diagnosis of relapsing GCA.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 4","pages":"718-727"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810613","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":"Cytotoxic CX3CR1+ T cells drive vascular inflammation in giant cell arteritis but not in Takayasu's arteritis.","authors":"Risa Inukai, Mitsuhiro Akiyama, Keiko Yoshimoto, Sohma Wakasugi, Yoshiyuki Matsuno, Sho Ishigaki, Waleed Alshehri, Koichi Saito, Yuko Kaneko","doi":"10.55563/clinexprheumatol/jmrl3k","DOIUrl":"10.55563/clinexprheumatol/jmrl3k","url":null,"abstract":"<p><strong>Objectives: </strong>To compare the involvement of cytotoxic CX3CR1+ T cell subsets between giant cell arteritis (GCA) and Takayasu's arteritis (TAK).</p><p><strong>Methods: </strong>We examined the proportions of CX3CR1+ CD4+ and CD8+ T cells in whole blood freshly obtained from 30 treatment-naive patients with active large vessel vasculitis (GCA, n=22 and TAK, n=8) and 16 healthy controls (HC). Infiltration of CX3CR1+ T cells into the affected arteries was assessed using immunohistochemical staining. Furthermore, CX3CR1+ CD4+ and CD8+ T cells were followed up after glucocorticoid treatment for longitudinal assessment of both diseases.</p><p><strong>Results: </strong>The proportion of CX3CR1+ CD4+ T cells was significantly higher in GCA than in HC but not in TAK. No differences were observed in the proportions of CX3CR1+ CD8+ T cells among the GCA, TAK, and HC groups. The increased proportion of CX3CR1+ CD4+ T cells in GCA strongly correlated with the severity of systemic inflammation, whereas no significant correlation was found in TAK. Compared to TAK, CX3CR1+ CD4+ T cells from GCA patients showed significantly higher expression of granzyme B and perforin. The inflamed temporal arterial tissues of the GCA were infiltrated by numerous CX3CR1+ T cells, contributing to inflammation, disruption of the elastic lamina, and intimal hyperplasia. In contrast, no infiltration of CX3CR1+ T cells was observed in the aortitis lesions of TAK. Longitudinal analysis of post-glucocorticoid treatment showed a reduction in CX3CR1+ T cells in GCA, whereas no significant change was observed in TAK.</p><p><strong>Conclusions: </strong>Differences in immune mechanisms between GCA and TAK highlight cytotoxic CX3CR1+ T cells as potential drivers for GCA-related inflammation and vessel damage but not for TAK.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"630-635"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496177","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":"International Congress on Large Vessel Vasculitis and Polymyalgia Rheumatica.","authors":"Christian Dejaco, Christina Duftner","doi":"10.55563/clinexprheumatol/ia22os","DOIUrl":"10.55563/clinexprheumatol/ia22os","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"775-798"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142557296","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":"Upper respiratory tract and orofacial manifestations of new-onset giant cell arteritis: results from a large, prospective inception cohort study.","authors":"Sébastien Laburthe, Kim-Heang Ly, Stéphanie Dumonteil, Nina Ratti, Edouard Desvaux, Guillaume Gondran, Holy Bezanahary, Sylvain Palat, Anne-Laure Fauchais, Eric Liozon, Simon Parreau","doi":"10.55563/clinexprheumatol/ryzoo7","DOIUrl":"10.55563/clinexprheumatol/ryzoo7","url":null,"abstract":"<p><strong>Objectives: </strong>Giant cell arteritis (GCA) often features upper respiratory tract (URT) and orofacial manifestations, which signal the involvement of external carotid artery branches. In this study, we aimed to describe the frequency of various URT/orofacial symptoms at GCA onset, as well as the main characteristics of patients presenting these symptoms.</p><p><strong>Methods: </strong>We included all patients who were newly diagnosed with GCA between 1976 and April 2022 at the Internal Medicine Department of a tertiary-care hospital. Ten URT or orofacial symptoms were prospectively examined systematically in each patient. We used multivariate analyses to identify the GCA characteristics, including URT/orofacial symptoms, associated with temporal artery biopsy (TAB) positivity.</p><p><strong>Results: </strong>At least 1 URT/orofacial symptom was present in 68.6% of the 599 patients (3 or more symptoms in 30% of cases). Jaw claudication, maxillary pain, and pain during mouth opening were the most prevalent symptoms. Dry cough was recorded in 17% of cases. GCA patients with URT/orofacial symptoms had more clinical abnormalities of the temporal artery bed and ischaemic ophthalmological complications, but less large-vessel vasculitis according to imaging. The likelihood of a positive TAB was increased in patients with an abnormal temporal artery upon clinical examination (OR 4.16; CI 2.75-6.37, p < 0.001) or jaw claudication (OR 2.18; CI 1.35-3.65, p = 0.002), and decreased in those with hoarseness (OR 0.47; CI 0.26-0.87, p = 0.02) or earache (OR 0.54; CI 0.31-0.95, p = 0.03). Isolated URT/orofacial presentation (i.e., without headache or visual signs) accounted for 5.2% of the entire cohort.</p><p><strong>Conclusions: </strong>Oral-facial symptoms were present in two-thirds of GCS cases. Thus, they could serve as leading clinical clues for a GCA diagnosis, and are a risk factor for permanent visual loss. Several URT/orofacial symptoms such as jaw claudication, hoarseness, and earache influenced the likelihood of a positive TAB. Isolated URT/orofacial presentation of GCA is a rare but potentially challenging occurrence.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"611-620"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141757405","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":"Clinical spectrum of small-vessel vasculitis related to cocaine consumption: data from an Italian cohort.","authors":"Silvia Grazzini, Edoardo Conticini, Alessia Giorli, Stefano Gentileschi, Luca Cantarini, Bruno Frediani","doi":"10.55563/clinexprheumatol/z41j3b","DOIUrl":"10.55563/clinexprheumatol/z41j3b","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"760-761"},"PeriodicalIF":3.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142124998","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}