Clinical and experimental rheumatology最新文献

{"title":"The co-occurrence of IgG4-related disease and malignancy: insights from a prospective cohort study.","authors":"Yufei Guo, Yiwen Wang, Minhui Lu, Hui Wang, Chao Xue, Xi Zheng, Wenrui Zhang, Yurong Zhao, Jie Zhang, Kunpeng Li, Jianglin Zhang, Feng Huang, Jian Zhu","doi":"10.55563/clinexprheumatol/5c7nti","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/5c7nti","url":null,"abstract":"<p><strong>Objectives: </strong>This study investigates the relationship between IgG4-related disease (IgG4-RD) and malignancies, focusing on tumour distribution and risk factors for malignancy development during follow-up.</p><p><strong>Methods: </strong>We analysed a prospective cohort of 278 IgG4-RD patients, including 22 with malignancies, and calculated standardised incidence ratios (SIRs). Bayesian statistical models were employed to identify risk factors.</p><p><strong>Results: </strong>Among 278 IgG4-RD patients, 22 (7.9%) developed malignancies. Lung cancer (13.6%) was the most prevalent malignancy, with a significantly higher incidence in the follow-up group compared to the history/concurrent group (19.3% vs. 7.0%; p=0.008). Testicular cancer occurred exclusively in the history/concurrent group (3.5%) and was absent in the follow-up group (p=0.044). The overall SIR was 3.28 (95% CI: 1.31-5.58) and lymphoma exhibited a markedly high SIR of 17.63 (95% CI: 0-52.89). Elevated ESR (estimate: 0.12, 95% CI: 0.04-0.23) and more organ involvements (estimate: 1.94, 95% CI: 0.31-3.93) were identified as significant risk factors for malignancy development.</p><p><strong>Conclusions: </strong>IgG4-RD patients exhibit an elevated risk of specific malignancies, particularly lymphoma, with elevated ESR and greater organ involvement identified as significant risk factors for malignancy development during follow-up period.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143968353","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":"Avacopan-induced liver injury: an uncommon but emerging concern?","authors":"Jia Sern, Keesha Ravintharan, Fahd Adeeb","doi":"10.55563/clinexprheumatol/o74k69","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/o74k69","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143986714","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":"Anifrolumab in the management of lupus headaches: a case report.","authors":"Pablo Martínez Calabuig, Jorge Juan Fragío Gil, Roxana González Mazarío, Laura Salvador Maicas, Mireia Lucía Sanmartín Martínez, Cristina Campos Fernández","doi":"10.55563/clinexprheumatol/c227y1","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/c227y1","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143966077","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":"Heterogeneity of peripheral immune cell landscape in systemic lupus erythematosus patients after belimumab treatment.","authors":"Wenchang Sun, Chunjuan Yang, Jin Zhang, Zhuojian Qu, Xinyue Hou, Na Liu, Jinghan Yang, Jiamei Sun, Lin Wang, Honggang Wang, Zengfang Wang, Donghua Xu","doi":"10.55563/clinexprheumatol/5i2xln","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/5i2xln","url":null,"abstract":"<p><strong>Objectives: </strong>The diversity and heterogeneity of circulating immune cells have been extensively investigated in systemic lupus erythematosus (SLE). However, little is known about the influence of belimumab, an anti-BAFF (B cell-activating factor) monoclonal antibody, on the heterogeneity of peripheral immune cell landscape in SLE patients. In this study, we aimed to investigate the altering effect of belimumab on autoimmunity in SLE.</p><p><strong>Methods: </strong>The single-cell RNA sequence revealed a total of 11 cell clusters by comparing the transcriptome profiles of 24.869 peripheral blood mononuclear cells (PBMCs) from normal controls (NC) and SLE patients with/without belimumab treatment. Flow cytometry was conducted to further confirm the diversity and heterogeneity of peripheral immune cell landscape. The disease-specific T cell, B cell, monocyte (M), and NK cell subpopulations in SLE patients treated with/without belimumab were identified.</p><p><strong>Results: </strong>Compared to the NC group, SLE patients exhibited a significant upregulation of CD3+T, CD8+T, CD3+PD-1+T, and CD8+PD-1+T cells, while the proportions of CD16+CD56+NK cell, CD14+CD206+ monocyte and CD14+CD163+ monocyte, and the ratio of CD3+CD4+T/CD3+CD8+T were significantly reduced in SLE. After belimumab treatment, the proportions of CD19+B and CD3+PD-1+T cells were significantly decreased in the peripheral blood of SLE patients.</p><p><strong>Results: </strong>Our study has implicated the substantial heterogeneity and disease-specific immune cell subsets in belimumab-treated and non-belimumab-treated SLE patients. Belimumab treatment may exert therapeutic effects in SLE patients probably by regulating the proliferation, phenotypes and functions of CD19+B cells and CD3+PD-1+T cells, which warrants further investigation in the future particularly regarding their potential roles and molecular mechanisms.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143975289","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":"Use of mycophenolate mofetil in new-onset systemic lupus erythematosus.","authors":"Hande Ogun, Sinem Nihal Esatoglu","doi":"10.55563/clinexprheumatol/fece6x","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/fece6x","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989602","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":"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":"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":"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":"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":"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}