Clinical and experimental rheumatology最新文献

{"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":"https://doi.org/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":""},"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":"Reply to the comment on: Beyond diagnosis: exploring the significance of IgG4+ plasma cell count through immunostaining in IgG4-related disease.","authors":"Eduardo Martín-Nares, Gabriela Hernández-Molina","doi":"10.55563/clinexprheumatol/0mrihy","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/0mrihy","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145198266","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":"Tracheobronchial stenosis in patients with granulomatosis with polyangiitis: histopathological findings and long-term outcome.","authors":"Louise Linde, Mikkel Faurschou, Sophine Boysen Krintel, Bo Baslund","doi":"10.55563/clinexprheumatol/xqt7su","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/xqt7su","url":null,"abstract":"<p><strong>Objectives: </strong>Tracheobronchial stenosis (TBS) occurs in a minority of patients with granulomatosis with polyangiitis (GPA). We aimed to i) review the literature on histopathological findings in patients with GPA and TBS, and ii) describe histopathological findings and long-term outcomes in a local cohort.</p><p><strong>Methods: </strong>PubMed was searched for articles including biopsy findings in patients with GPA and TBS until May 2023. Our local cohort included patients diagnosed with GPA and TBS during 1991-2022. Demographic and clinical characteristics, number of biopsies, histopathological findings (GPA-characteristic versus non-specific) and number of endoscopic dilations during follow-up were recorded.</p><p><strong>Results: </strong>In the reviewed literature, 203 patients were identified; 128 (63%) had a total of 296 biopsies; 64 (22%) with GPA-characteristic findings. The local cohort comprised 27 patients, the median (range) age at GPA diagnosis was 31 (14-76) years, the follow-up time was 17 (0-31) years. TBS occurred 2 (0-21) years after the GPA diagnosis. In the local cohort, 67 biopsies from 26 patients were available. GPA-characteristic findings were seen in 15 (22%) of these. Twenty-three patients underwent an endoscopic dilation during follow-up, and the number of dilations was 5 (1-100). Eighteen patients had ≥2 dilations; the interval between dilations was 23 (2-103) months.</p><p><strong>Conclusions: </strong>The majority of patients with GPA and TBS experience recurring airway stenoses. GPA-characteristic findings are seen in approximately one in five tracheobronchial biopsies obtained from GPA patients with TBS. A biopsy could be of clinical value in the diagnostic process or to exclude other causes of stenosis.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145198298","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":"GITR/GITRL interaction promotes the expansion of T helper 9 and T helper 17 in psoriatic arthritis.","authors":"Lidia La Barbera, Chiara Rizzo, Marianna Lo Pizzo, Diana Di Liberto, Marco Pio La Manna, Leila Mohammadnezhad, Federica Camarda, Guido Sireci, Francesco Ciccia, Giuliana Guggino","doi":"10.55563/clinexprheumatol/31hjvf","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/31hjvf","url":null,"abstract":"<p><strong>Objectives: </strong>Psoriatic arthritis (PsA) is a chronic inflammatory disease characterised by the involvement of multiple targets. Accumulating evidence suggests the key role played by T helper (Th)9 and Th17 cells in PsA. Recently, the ability to activate GITR in promoting differentiation and proliferation of Th17 and Th9 cells has been investigated in several inflammatory conditions. We aimed to evaluate the effects of GITR/GITRL interaction in the immune responses underlying the disease, including the main PsA target sites.</p><p><strong>Methods: </strong>Twenty-one PsA patients with active disease, naive to disease-modifying anti-rheumatic drugs, were enrolled. Peripheral blood mononuclear cells and synovial fluid (SF) mononuclear cells were collected to assess GITR and GITRL expression by flow cytometry. An in vitro functional assay with recombinant GITR agonist was performed to detect the effect on T cell subsets. Synovial and ileal biopsies were obtained to evaluate GITR and GITRL expression by immunofluorescence. Healthy subjects and osteoarthritis patients were enrolled as controls.</p><p><strong>Results: </strong>We reported an increased in vitro expression of GITR among CD4+ T cells and its cognate ligand GITRL on antigen-presenting cells in PsA peripheral blood. In vitro, the addition of the GITR agonist resulted in increased expansion of Th9 and Th17 cells. Increased expression of GITR and GITRL was found even in PsA SF, synovium and ileum.</p><p><strong>Conclusions: </strong>Our results suggest a novel role of GITR/GITRL in promoting the expansion of Th9 and Th17 in PsA-inflamed tissues.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145173971","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":"Obesity in primary Sjögren's disease.","authors":"Ivette Cruz-Bautista, Donaji Gómez-Velasco, Ariel García-Torralba, Carlos A Aguilar-Salinas, Gabriela Hernandez-Molina","doi":"10.55563/clinexprheumatol/9d0mr2","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/9d0mr2","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate the prevalence of obesity in primary Sjögren's disease (SjD), and assess its association with clinical/serologic features, disease activity, damage, and sicca symptoms.</p><p><strong>Methods: </strong>Transversal study that included 91 patients. We registered demographics, comorbidities, glandular/extra-glandular and serologic variables. We assessed the Schirmer-I test and non-stimulated salivary flow, and scored the cumulative ESSDAI, SSDDI and ESSPRI scores. We measured the body mass index (BMI), waist circumference (WC) and waist-to-hip ratio (WHR). We defined obesity as a BMI ≥30 kg/m2. Central obesity was defined as WC >90 cm and >80 cm; or by a WHR >0.90 and >0.85, for men and women, respectively. All patients underwent bioimpedance analysis to measure body fat mass index (FMI). An elevated/high FMI was classified as obese.</p><p><strong>Results: </strong>According to BMI, 18 patients were obese (19.7%), while 33 (36.2%) were obese according to WC, 48 (52.7%) according to WHR, and 37(40.6%) according to FMI. When we compared obese vs non-obese patients according to BMI, the first group had a higher prevalence of anti-Ro/SSA antibodies. When we then performed the same groups comparison, but now using the WC, WHR and FMI definitions, the multivariate analysis showed an association between SSDDI and obesity.</p><p><strong>Conclusions: </strong>According to BMI, at least 20% of patients were obese, this prevalence increased to 40% when BIA was used, with a higher prevalence found in central obesity. Obesity did not impact the symptoms and disease activity but might be associated with damage. Our results may have implications for weight reduction in these patients.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145173981","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":"Comment on: Triple-combination therapy did not improve prognosis in anti-MDA5 positive dermatomyositis: a multicentre longitudinal cohort study by You et al.","authors":"Wilmer Rojas-Zuleta","doi":"10.55563/clinexprheumatol/spyyh8","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/spyyh8","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145174030","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":"Data from a multicentric international study unveils a neglected association between chronic non-bacterial osteomyelitis and inflammatory bowel diseases.","authors":"Caterina Matucci-Cerinic, Yagmur Bayindir, Chiara Longo, Francesco Mori, Francesca Alessi, Matteo Vergani, Silvia Rosina, Alessandro Consolaro, Stefano Volpi, Roberta Caorsi, Serena Arrigo, Marco Gattorno, Seza Ozen, Clara Malattia","doi":"10.55563/clinexprheumatol/2pobji","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/2pobji","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to evaluate the clinical, serological, radiological characteristics and response to treatments of patients with chronic non-bacterial osteomyelitis (CNO) associated with inflammatory bowel diseases (C-IBD) in comparison to CNO patients without gastrointestinal disease.</p><p><strong>Methods: </strong>Patients with C-IBD followed in the Rheumatology Department of the Istituto Gaslini, Genova, and Hacettepe Hospital, Ankara, were retrospectively enrolled in the Eurofever Registry and compared to a group of CNO patients. A literature review on paediatric C-IBD was also performed.</p><p><strong>Results: </strong>19 C-IBD patients were compared to 57 CNO. In C-IBD, disease onset was characterised by osteoarticular symptoms in 73.6%, by gastrointestinal symptoms in 10.5%, and was simultaneous in 15.8%. Spinal involvement was more frequent in CNO (p<0.05), while sacroiliac involvement was more frequent in C-IBD (p<0.005). 42% of C-IBD presented low-grade fever (p<0.05, 15% of CNO). All C-IBD presented a CRP elevation, present in only 45% of CNO (p<0.0001). In 68.4% of C-IBD, CRP remained elevated and became negative only after IBD treatment was started. Moreover, 58% of C-IBD patients presented microcytic anaemia (present in 17.6% CNO, p=0.0005). Fecal calprotectin resulted positive in 100% of C-IBD and 10% of CNO (p<0.0001).</p><p><strong>Conclusions: </strong>This is the largest cohort of paediatric C-IBD reported in the literature. The persistence of inflammation and the presence of microcytic anaemia were associated with the development of an IBD. Given the frequent absence of intestinal symptoms, screening with fecal calprotectin is suggested in all CNO patients that present a persistent CRP elevation despite treatment, and have microcytic anaemia.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145174001","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: Use of upadacitinib in giant cell arteritis.","authors":"Daniel Blockmans, Weihan Zhao, Arathi R Setty, Peter A Merkel","doi":"10.55563/clinexprheumatol/ozaczo","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/ozaczo","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145173995","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":"https://doi.org/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":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-24","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":"Circulating microRNAs associated with tumour necrosis factor or fibloblast-like synoviocyte predict cartilage damage in early rheumatoid arthritis treated with methotrexate plus adalimumab: a subanalysis of the MIRACLE study.","authors":"Yohei Hosokawa, Yusuke Yoshida, Hiroya Tamai, Shintaro Hirata, Kei Ikeda, Toshiaki Miyamoto, Hiroaki Taguchi, Chang-Fu Kuo, Kichul Shin, Keisuke Izumi, Yasushi Kondo, Hidekata Yasuoka, Masataka Kuwana, Tomonori Ishii, Hideto Kameda, Toshihisa Kojima, Masahiko Mori, Yasunori Sato, Wen-Chan Tsai, Tsutomu Takeuchi, Yuko Kaneko","doi":"10.55563/clinexprheumatol/keymab","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/keymab","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to explore the potential of plasma micro-ribonucleic acids (miRNAs) in predicting joint damage in patients with rheumatoid arthritis (RA).</p><p><strong>Methods: </strong>This subanalysis of the MIRACLE study, a randomised, open-label, non-inferiority trial, explored and compared the efficacy and safety of treatment with adalimumab (ADA), an anti-tumour necrosis factor (TNF) α, plus a maximum tolerated dose of methotrexate (MTX) with a reduced dose of MTX in early RA. Plasma levels of miRNAs (miR-143-3p, miR-146a-5p, miR-155-5p, miR-182-5p, miR-21-5p, and miR-221-3p) and serum levels of inflammatory cytokines (interleukin-6 [IL-6], vascular endothelial growth factor [VEGF]) and matrix metalloprotease-3 (MMP-3) were measured at 24 weeks. Their association with joint destruction assessed by the modified total Sharp score [mTSS] over the 24-week period were analysed.</p><p><strong>Results: </strong>A total of 134 patients who showed an inadequate response to MTX and started treatment with ADA were included in the analyses. Logistic regression analyses revealed that higher plasma levels of miR-143-3p, miR-146a-5p, miR-21-5p, and miR-221-3p were significantly associated with increases in mTSS >0.5 points during the observation period. In particular, positive correlation was derived from the progression of joint space narrowing. In contrast, MMP-3, VEGF, and IL-6 levels were not associated with joint destruction. Cartilage damage occurred mainly in patients treated with reduced dose of MTX.</p><p><strong>Conclusions: </strong>Higher circulating miRNA levels predicted subsequent cartilage damage in early RA treated with a TNF inhibitor in addition to MTX. Thus, the MTX dose at ADA initiation should not be reduced in patients with high microRNA levels.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145174021","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}