{"title":"Validity of the 2023 ACR/EULAR APS classification criteria to pediatric primary APS: inputs from a systematic literature review.","authors":"Stefano Romano, Edoardo Marrani, Valerio Maniscalco, Teresa Giani, Ilaria Pagnini, Maria Vincenza Mastrolia, Ilaria Maccora, Giulia Carreras, Gabriele Simonini","doi":"10.1093/rheumatology/keag586","DOIUrl":"https://doi.org/10.1093/rheumatology/keag586","url":null,"abstract":"<p><strong>Objectives: </strong>The 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria have been introduced and validated in adult populations; however, data on their performance in pediatric APS remain limited. The present study aims to apply the 2023 EULAR/ACR classification criteria to a cohort of pediatric patients with primary APS identified through a systematic review of the literature.</p><p><strong>Methods: </strong>A systematic literature review was conducted to identify primary pediatric APS. Individual patient data were extracted. A two-stage individual participant data-style random-effects meta-analysis was performed to estimate the pooled sensitivity of the APS scoring systems.</p><p><strong>Results: </strong>A total of 118 studies were included, comprising 148 pediatric patients with primary APS. Thrombotic events were reported in 93.2% of patients, while non-thrombotic manifestations occurred in 56.8%; the most frequent extra-thrombotic manifestation was thrombocytopenia (29%). Overall, the 2006 Sapporo criteria reached a sensitivity of 91.2% (95% CI: 85.4-95.2), while the 2023 ACR/EULAR APS classification criteria of 83.1% (95% CI: 76.2-88.3). Most patients who did not satisfy the new criteria set failed to meet the laboratory domains.</p><p><strong>Conclusion: </strong>The 2023 ACR/EULAR APS classification criteria demonstrate suboptimal sensitivity when applied to pediatric primary APS. While the inclusion of non-thrombotic manifestations improved the classification of pediatric APS patients, limitations remain, particularly regarding laboratory antiphospholipid antibody thresholds and the exclusion of some pediatric-relevant clinical features, confirming the incomplete overlap between pediatric and adult-onset APS. These findings highlight the need for pediatric-specific APS classification criteria to improve disease characterization and research in this population.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149231181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-20DOI: 10.1093/rheumatology/keag591
Clemens Untersulzner, Andreas Kronbichler
{"title":"Cardiovascular risk in ANCA-associated vasculitis: a plea for early intervention.","authors":"Clemens Untersulzner, Andreas Kronbichler","doi":"10.1093/rheumatology/keag591","DOIUrl":"https://doi.org/10.1093/rheumatology/keag591","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reasons-stratified treatment retention after JAK inhibitor discontinuation in rheumatoid arthritis: the ANSWER cohort study.","authors":"Yuhei Fujisawa, Ryu Watanabe, Yuya Yano, Kosaku Murakami, Koichi Murata, Yuri Hiramatsu, Toru Takeuchi, Yuki Etani, Yasutaka Okita, Hideki Amuro, Hirotaka Yamada, Yo Ueda, Ryota Hara, Masaki Katayama, Yuji Nozaki, Tetsu Itami, Wataru Yamamoto, Ryuhei Ishihara, Masao Katsushima, Kazuo Fukumoto, Shinsuke Yamada, Tadashi Okano, Motomu Hashimoto","doi":"10.1093/rheumatology/keag587","DOIUrl":"https://doi.org/10.1093/rheumatology/keag587","url":null,"abstract":"<p><strong>Objective: </strong>To compare the retention and disease activity trajectories of subsequent biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) after Janus kinase inhibitor (JAKi) discontinuation in patients with rheumatoid arthritis (RA).</p><p><strong>Methods: </strong>We analysed 500 treatment courses (TCs) from the ANSWER cohort in which subsequent b/tsDMARDs were initiated after JAKi discontinuation. Subsequent treatments were classified as TNF inhibitors (TNFi), anti-IL-6 receptor antibodies (aIL-6R), CTLA4-Ig and JAKi. Drug retention was stratified by the reason for prior JAKi discontinuation and evaluated using Kaplan-Meier and multivariable Cox analyses. Changes in Clinical Disease Activity Index (CDAI) over 12 months were analysed using mixed-effects models for repeated measures.</p><p><strong>Results: </strong>After prior JAKi discontinuation due to ineffectiveness, CTLA4-Ig was associated with a significantly higher risk of discontinuation due to ineffectiveness than subsequent JAKi (HR 2.83, 95% CI 1.20-6.70). Among TCs in which the prior JAKi was discontinued due to toxic adverse events (AEs), TNFi and aIL-6R were associated with lower risks of toxic AE-related discontinuation than JAKi. Overall CTLA4-Ig was associated with higher risks of discontinuation due to ineffectiveness (HR 2.57, 95% CI 1.46-4.54) and total discontinuation excluding non-toxic reasons and remission (HR 1.77, 95% CI 1.02-3.07). CDAI trajectories over 12 months did not differ significantly between treatment groups.</p><p><strong>Conclusion: </strong>The optimal treatment strategy after JAKi discontinuation may depend on the reason for prior discontinuation. JAKi cycling, TNFi and aIL-6R may be reasonable after ineffectiveness, whereas after toxic AEs, TNFi and aIL-6R were associated with lower risks of subsequent toxic AE-related discontinuation than JAKi cycling.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228680","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-20DOI: 10.1093/rheumatology/keag590
Marc Xipell, Maria C Garbarino, Ignasi Rodríguez-Pintó, Estibaliz Ruiz-Ortiz, Avinash Chandu, Juan D Díaz-García, Adriana García-Herrera, José A Gómez-Puerta, Juan C Sarmiento-Monroy, Ricard Cervera, Arturo Pereira, Gerard Espinosa, Luis F Quintana
{"title":"Deep clinical renal response in lupus nephritis treated with belimumab: a real-world cohort study.","authors":"Marc Xipell, Maria C Garbarino, Ignasi Rodríguez-Pintó, Estibaliz Ruiz-Ortiz, Avinash Chandu, Juan D Díaz-García, Adriana García-Herrera, José A Gómez-Puerta, Juan C Sarmiento-Monroy, Ricard Cervera, Arturo Pereira, Gerard Espinosa, Luis F Quintana","doi":"10.1093/rheumatology/keag590","DOIUrl":"https://doi.org/10.1093/rheumatology/keag590","url":null,"abstract":"<p><strong>Objectives: </strong>Lupus nephritis (LN) often progresses despite therapy, and current complete renal response (CRR) definitions tolerate residual proteinuria deemed suboptimal in other glomerulopathies. Belimumab, the first biologic approved for active LN, may enable deeper remission, but its real-world effectiveness is unknown.</p><p><strong>Methods: </strong>We retrospectively studied a single-centre cohort of biopsy-proven LN receiving add-on belimumab (2015-2025), with follow-up to 24 months and a paired-biopsy subgroup. We explored a study-specific stringent composite endpoint, deep clinical renal response (DCRR): urine protein-to-creatinine ratio (uPCR) <200 mg/g, absence of active glomerular haematuria and stable renal function, benchmarked against conventional CRR (uPCR <500 mg/g, stable renal function).</p><p><strong>Results: </strong>Of 51 patients, 43 not in CRR at initiation were analysed. DCRR was achieved by 38.5% at 12 and 53.8% at 24 months, versus 66.7% and 76.9% for CRR, in available-case analysis; deep responses accrued gradually (median 17.7 vs 7.5 months), still increasing during the second year. Baseline uPCR was the only variable consistently associated with 12-month response. Renal response occurred alongside improvements in systemic activity, serological markers, and glucocorticoid exposure (SLEDAI-2K 13 to 2; anti-dsDNA 205 to 54 IU/mL and glucocorticoid reduction in 72%). In eight paired biopsies, activity index fell from 5.5 to 2 (p = 0.016) with attenuated glomerular C3 deposition (3+ to 1+, p = 0.031), whereas chronicity indices rose; these findings are exploratory given the small, uncontrolled sample.</p><p><strong>Conclusion: </strong>Deep renal response, accompanied by regression of histological activity, appears achievable in a growing proportion of patients. However, its impact on long-term renal protection remains to be established.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-20DOI: 10.1093/rheumatology/keag584
Francesco Zulian, Francesca Tirelli, Biagio Castaldi, Elisabetta Zanatta, Fabio Vittadello, Greta Mastrangelo, Alessandra Meneghel
{"title":"Distinct trajectories of disease severity progression in juvenile systemic sclerosis.","authors":"Francesco Zulian, Francesca Tirelli, Biagio Castaldi, Elisabetta Zanatta, Fabio Vittadello, Greta Mastrangelo, Alessandra Meneghel","doi":"10.1093/rheumatology/keag584","DOIUrl":"https://doi.org/10.1093/rheumatology/keag584","url":null,"abstract":"<p><strong>Objective: </strong>Juvenile systemic sclerosis (JSSc) often presents with overlapping features, making phenotypic classification and disease progression difficult to define. This study investigated whether JSSc comprises latent disease severity subgroups with distinct longitudinal trajectories and examined baseline predictors of class membership.</p><p><strong>Methods: </strong>Retrospective cohort study using prospectively collected data from 45 patients with JSSc between January 2005 and December 2024. Demographic, clinical, laboratory, and disease severity data were collected. The primary outcome was progression-free survival (PFS), defined as percentage change in the Juvenile Systemic Sclerosis Severity score (J4S) over time. A Growth Mixture Model identified latent disease severity classes with distinct longitudinal trajectories and their baseline predictors.</p><p><strong>Results: </strong>Thirty-eight JSSc patients (66% females), followed for ≥48 months, entered the study. PFS remained high in the fibrotic and limited cutaneous JSSc subtypes (80-100%) over the observation period, whereas the diffuse and sine scleroderma subtypes showed marked deterioration, with PFS decreasing to 54% and 25%, respectively. We identified two latent classes of disease severity: Class 1 showed low severity and good treatment response, Class 2 showed an active, progressive course with poor long-term outcomes. Baseline predictors of poor outcome were J4S >7.5, treatment initiation >9 months from disease onset, and >1.5 organs involved. Latent trajectory classes showed good concordance with JSSc clinical subtypes (κ = 0.660).</p><p><strong>Conclusion: </strong>We identified two latent classes of disease severity in JSSc with clear prognostic significance. High baseline J4S, delayed treatment, and greater organ involvement predicted a more severe disease course, paving the way for precision health strategies and optimized.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228606","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-20DOI: 10.1093/rheumatology/keag562
Yuji Kamiyama, Tatsuma Ban, Go R Sato, Masako Kikuchi, Tomo Nozawa, Seira Hattori, Kenichi Nishimura, Tomohiko Tamura, Shuichi Ito
{"title":"A case of STING-associated vasculopathy with onset in infancy highlighting the pathogenetic role of interferon regulatory factor 5.","authors":"Yuji Kamiyama, Tatsuma Ban, Go R Sato, Masako Kikuchi, Tomo Nozawa, Seira Hattori, Kenichi Nishimura, Tomohiko Tamura, Shuichi Ito","doi":"10.1093/rheumatology/keag562","DOIUrl":"https://doi.org/10.1093/rheumatology/keag562","url":null,"abstract":"<p><strong>Objectives: </strong>To elucidate the pathophysiology of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), we evaluated interferon-stimulated gene (ISG) expression and nuclear translocation of interferon regulatory factors 3 and 5 (IRF3 and IRF5) in patient-derived cells before and after Janus kinase (JAK) inhibitor treatment.</p><p><strong>Methods: </strong>A 3-year-old girl presenting with refractory eczema, polyarthritis, and interstitial lung disease (ILD) was confirmed to have SAVI. Baricitinib, a JAK1/2 inhibitor, was initiated for refractory ILD, and peripheral blood samples were analysed before and 4 months after baricitinib initiation. Expression of representative ISGs (IFI27, MX1, and OAS1) was quantified by reverse-transcription quantitative polymerase chain reaction. Nuclear localization of IRF3 and IRF5 was examined in sorted monocytes and B cells using confocal microscopy after intracellular staining. Activation was quantified by the nuclear-to-cytoplasmic mean fluorescence intensity ratio.</p><p><strong>Results: </strong>ISG expression was markedly elevated at baseline and decreased to levels comparable to those in a healthy control subject after 4 months of treatment. IRF3 localization remained unchanged, whereas IRF5 showed pronounced nuclear enrichment in monocytes at baseline, an effect that diminished after treatment. This reduction paralleled clinical improvement, including resolution of lung involvement and rash, and seroreversion of autoantibodies.</p><p><strong>Conclusion: </strong>IRF5 activation may sustain chronic inflammation in SAVI independently of IRF3. The observed reduction in IRF5 nuclear translocation paralleled the improvement in disease activity, suggesting that targeting IRF5 may help suppress inflammatory processes not adequately controlled by existing treatments.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-20DOI: 10.1093/rheumatology/keag588
Sara K Tedeschi
{"title":"Contemporary issues relating to calcium pyrophosphate deposition disease.","authors":"Sara K Tedeschi","doi":"10.1093/rheumatology/keag588","DOIUrl":"https://doi.org/10.1093/rheumatology/keag588","url":null,"abstract":"<p><p>Calcium pyrophosphate deposition (CPPD) disease is a common crystalline arthritis that has historically been under-studied compared to other rheumatic diseases including gout. For decades, the presence of CPPD in joints has been consider of uncertain clinical importance, with conflicting reports on whether CPPD leads to osteoarthritis progression or other adverse outcomes. A growing body of literature in the past five years has challenged the prevailing view of CPPD disease as \"just chondrocalcinosis.\" These findings have arrived together with recent developments in classifying CPPD disease for research studies, advances in quantification of CPPD on imaging studies, consensus on outcomes to be measured in CPPD disease clinical trials, newly-identified genetic associations, and updates in the nomenclature for this crystalline arthritis. This review summarizes key updates in each of these realms.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149228675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-19DOI: 10.1093/rheumatology/keag558
Claire Adams, Marie-Eve Carrier, Meira Golberg, Natalie Co, Elsa-Lynn Nassar, Linda Kwakkenbos, Susan J Bartlett, Catherine Fortuné, Amy Gietzen, Geneviève Guillot, Amanda Lawrie-Jones, Vanessa L Malcarne, Maureen D Mayes, Michelle Richard, Luc Mouthon, Andrea Benedetti, Brett D Thombs
{"title":"Health literacy among people with systemic sclerosis: a scleroderma patient-centred intervention network (SPIN) cohort cross-sectional study.","authors":"Claire Adams, Marie-Eve Carrier, Meira Golberg, Natalie Co, Elsa-Lynn Nassar, Linda Kwakkenbos, Susan J Bartlett, Catherine Fortuné, Amy Gietzen, Geneviève Guillot, Amanda Lawrie-Jones, Vanessa L Malcarne, Maureen D Mayes, Michelle Richard, Luc Mouthon, Andrea Benedetti, Brett D Thombs","doi":"10.1093/rheumatology/keag558","DOIUrl":"https://doi.org/10.1093/rheumatology/keag558","url":null,"abstract":"<p><strong>Objective: </strong>Health literacy reflects the ability to find, understand, evaluate, and use information to make health-related decisions. We evaluated sociodemographic and disease factors associated with health literacy among people with systemic sclerosis (SSc).</p><p><strong>Methods: </strong>Participants with SSc in the Scleroderma Patient-centred Intervention Network Cohort completed the Health Literacy Scale19-12 Item Questionnaire from February to November 2025. Multivariable linear regression was used to assess associations of sociodemographic and disease characteristics with health literacy.</p><p><strong>Results: </strong>The 1,019 participants were mainly female (N = 898, 88%) and White (N = 850, 84%). Mean (SD) years of education was 15.5 (3.9). Most had limited cutaneous SSc (N = 669, 66%). Mean health literacy total score was 69.7; 438 (43%) participants had excellent or sufficient and 581 (57%) had problematic or inadequate health literacy. Education (1.63 points per 5 years, 95% CI 0.25 to 3.01) was significantly associated with higher health literacy scores. Compared to the United States, participants from Canada (-4.98 points, 95% CI -7.99 to -1.97), the United Kingdom (-7.79 points, 95% CI -12.03 to -3.55), France (-6.05 points, 95% CI -8.84 to -3.27), and Australia, Mexico, or Spain (-7.02 points, 95% CI -12.39 to -1.66) had significantly lower health literacy scores. Age, gender, race or ethnicity, marital status, time since first non-Raynaud symptom onset, and disease subtype were not associated with health literacy.</p><p><strong>Conclusion: </strong>The association between health literacy and education was small. Differences between countries may reflect self-evaluation tendencies. More work is needed to better understand health literacy measurement.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RheumatologyPub Date : 2026-10-19DOI: 10.1093/rheumatology/keag563
Shuang Xu, William F Jiemy, Nanthicha Inrueangsri, YouBeen Ko, Wayel H Abdulahad, Ellen Verschoor, Yannick van Sleen, Jacoba C Graver, Kornelis S M van der Geest, Gwenny M Verstappen, Elisabeth Brouwer, Peter Heeringa, Annemieke M H Boots, Maria Sandovici
{"title":"Activated B cells steer fibroblasts towards a proinflammatory phenotype in giant cell arteritis.","authors":"Shuang Xu, William F Jiemy, Nanthicha Inrueangsri, YouBeen Ko, Wayel H Abdulahad, Ellen Verschoor, Yannick van Sleen, Jacoba C Graver, Kornelis S M van der Geest, Gwenny M Verstappen, Elisabeth Brouwer, Peter Heeringa, Annemieke M H Boots, Maria Sandovici","doi":"10.1093/rheumatology/keag563","DOIUrl":"https://doi.org/10.1093/rheumatology/keag563","url":null,"abstract":"<p><strong>Objectives: </strong>Giant cell arteritis (GCA) is characterized by chronic large-vessel inflammation and remodelling. Within affected arteries, especially in aorta, organized B cell clusters are mainly localized in adventitia, where fibroblasts represent the dominant stromal population. This study aims to investigate fibroblast heterogeneity within these clusters and B cell-fibroblast interactions in vitro as possible contributors to disease chronicity in GCA.</p><p><strong>Methods: </strong>Fibroblast phenotypes in CD20+B cell clusters in GCA and atherosclerotic aortic tissues were characterized using immunohistochemistry for fibroblast markers (CD90, PDPN, FAP, CD248, α-SMA) and cytokine expression (IL-6, GM-CSF, BAFF). In vitro, peripheral blood B cells from treatment-naïve GCA patients and healthy donors were stimulated and cocultured with human aortic adventitial fibroblasts (HAoAFs) using either direct cell coculture or transwell systems. To identify relevant molecular pathways for B cell-fibroblast interaction, HAoAFs were pre-treated with infliximab, tocilizumab, baricitinib or upadacitinib, followed by exposure to activated B cell conditioned medium (aBCCM). Protein secretion was assessed by ELISA, Luminex and Olink, mRNA expression was determined by qPCR.</p><p><strong>Results: </strong>CD90+PDPN+immunofibroblasts mainly localized centrally, whereas CD90+FAP+ and CD90+CD248+fibroblasts mostly located at the border of the clusters. Fibroblasts in/around clusters co-expressed IL-6, GM-CSF, and BAFF. In vitro, HAoAFs cocultured with activated B cells or supernatants thereof upregulated IL-6, GM-CSF, PDPN, and FAP mRNA, and actively secreted IL-6, GM-CSF and BAFF, while type I collagen expression was suppressed. JAK-STAT inhibition abrogated these pro-inflammatory effects, whereas TNF-α and IL-6 receptor blockade did not.</p><p><strong>Conclusion: </strong>Activated B cells drive fibroblasts toward a pro-inflammatory phenotype, potentially sustaining chronic inflammation and disease progression in GCA. JAK-STAT signalling appears central to this effect, highlighting its therapeutic potency at the vascular level in GCA.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2026-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149224748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}