Rheumatology最新文献

{"title":"NK cell cytotoxicity is markedly reduced in younger patients with rheumatoid arthritis treated with JAK inhibitors","authors":"Takahiko Akagi, Yasumitsu Nishimura, Shunichi Fujita, Hiroyasu Hirano, Akiko Nagasu, Shoko Tsuji, Yoshitaka Morita, Kazuhisa Nakano","doi":"10.1093/rheumatology/keaf308","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf308","url":null,"abstract":"Objectives This study investigated the impact of Janus kinase inhibitors (JAKi) on natural killer (NK) cell activity and phenotype in patients with rheumatoid arthritis (RA). While JAKi are effective in achieving low disease activity (LDA), preclinical and pharmacokinetic studies suggest potential effects on NK cells, raising concerns about infection and malignancy risks. However, the extent of these effects in RA patients remains unclear. Methods We assessed the cytotoxic activity of peripheral blood NK cells in RA patients with LDA who had been treated with JAKi (n = 85) or TNF inhibitors (TNFi) (n = 131) for more than three months using a 51Cr-release assay. Propensity score matching was used to adjust for confounders, and multivariable logistic regression identified risk factors for low NK cell activity. NK cell maturation and differentiation were evaluated via flow cytometry. Results NK cell activity was significantly lower in the JAKi group compared to the TNFi group (median 50% vs 36%, p = 0.002). The proportion of NK and CD56dim cells was reduced, while CD56bright cells were more abundant in the JAKi group. In the JAKi group, glucocorticoid use (odds ratio [OR] 5.36, 95% confidence interval [CI] 1.10-26.07, p = 0.04) and younger age (<50 years; OR 10.56, 95% CI 2.69-41.45, p < 0.01) were independent risk factors for reduced NK cell activity. Notably, herpes zoster incidence was significantly higher in the low NK cell activity group among RA patients younger than 50 years. Conclusion JAKi treatment significantly impaired NK cell activity and differentiation in RA patients, with age influencing these effects, underscoring important safety concerns.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"331 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144218938","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":"Risk of infection in patients with early inflammatory arthritis: results from a large UK prospective observational cohort study","authors":"Maryam A Adas, Katie Bechman, Mark D Russell, Victoria Allen, Samir Patel, Mark Gibson, Ioasaf Karafotias, Kathryn Biddle, Benjamin Zuckerman, Kaiyang Song, Deepak Nagra, Edward Alveyn, Suma Mahendrakar, Meryem Nursoy, Fabiola Atzeni, Sarah Gallagher, Elizabeth Price, Mark Garton, Andrew Rutherford, Andrew P Cope, Sam Norton, James B Galloway","doi":"10.1093/rheumatology/keaf312","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf312","url":null,"abstract":"Objective To identify risk of serious infections-(SI) according to initial conventional synthetic disease modifying anti-rheumatic drugs-(csDMARD) and corticosteroids, in patients recruited to the National Early Inflammatory Arthritis Audit. Methods An observational cohort study was used, including adults in England and Wales with new diagnoses of rheumatoid arthritis-(RA) between 2018–2023. Main outcome was SI-events, defined as infections requiring hospitalisation/or resulting in death. Secondary analyses evaluated SI-related mortality alone. Hazard ratios-(HR) were calculated using cox proportional hazards models. Primary predictor was initial treatment strategy, with confounder adjustments. Results 17 472 patients were included, of whom 10 997 on methotrexate-based strategies; 4,540 on other csDMARDs; 13 680 received corticosteroids. There were 1307 SI-events, corresponding to incidence rates per 100 person-years of 3.02 (95% CI: 2.86–3.19) and 311 SI-related mortality (IR 0.69, 95% CI: 0.61–0.77). Methotrexate-based strategies were associated with reduced risk of SI-events compared with other csDMARDs (adjusted HR 0.72, 95% CI: 0.63–0.82). In unadjusted models, corticosteroid was associated with higher risk of SI-events, but in adjusted models this association was no longer significant (adjusted HR 0.99, 95% CI: 0.87–1.12). Increasing age, being a current/or ex-smoker (relative to non-smoker), having a comorbidity, being seropositive, and having high DAS28 all associated with increased incidence of SI. One unit increase in baseline DAS28 increases the risk of SI-event by 10%. Conclusion Methotrexate-based regimens associated with a reduced risk of SI compared with other strategies. Patient-level and disease-related factors at diagnosis are important predictors of SI in individuals with new RA.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"42 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144228590","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":"High levels of EPSTI1 enhance IFN-β-mediated HLA-A expression and chemokine secretion in myoblasts in dermatomyositis.","authors":"Xiaojing Li, Ting Ding, Yizhi Xiao, Junyu Zhou, Ting Huang, Shasha Xie, Qiming Meng, Weijia He, Hongling Zhu, Hui Luo","doi":"10.1093/rheumatology/keaf297","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf297","url":null,"abstract":"<p><strong>Objectives: </strong>Epithelial-Stromal Interaction 1 (EPSTI1), an interferon-related gene that has emerged as a gene of notable interest, plays a multifaceted role in cellular function and disease processes. However, the precise role of EPSTI1 in the context of dermatomyositis(DM) remains elusive and requires further exploration.</p><p><strong>Methods: </strong>To investigate EPSTI1 expression in DM, we analyzed two transcriptome datasets, peripheral blood mononuclear cells (PBMCs) and muscle tissues from our DM cohort. We further validated the findings using RT-qPCR and immunohistochemical staining within our idiopathic inflammatory myopathy (IIM) cohort. Through Small Interfering RNA (siRNA)-mediated knockdown, we delved into the function and underlying mechanisms of EPSTI1 in DM patients.</p><p><strong>Results: </strong>EPSTI1 is significantly upregulated in both PBMCs and muscle tissues of DM patients. A notable positive correlation between EPSTI1 expression and interferon-stimulating genes (ISGs) accompanies this upregulation. Furthermore, EPSTI1 levels were markedly elevated in interferon-β(IFN-β)-stimulated myoblasts. Functional studies have shown that downregulation of EPSTI1 inhibits multiple immune and inflammatory pathways, including antigen processing and presentation, chemokine-mediated signalling, interferon signalling, IL-1-mediated signalling, NIK/NF-κB signalling. EPSTI1 was involved in the expression of HLA-A and the secretion of chemokines in myoblasts mediated by IFN-β.</p><p><strong>Conclusion: </strong>EPSTI1 might play an essential role in promoting muscle inflammation in DM by regulating the expression of HLA-A and the secretion of chemokines in myoblasts. Targeting EPSTI1 may represent a novel therapeutic approach for reducing muscle inflammation and damage in DM patients.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209393","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":"Long-term effects of selexipag in systemic sclerosis-associated digital ulcers: a case control multicentre observational study.","authors":"Claudia Iannone, Marco Di Battista, M R Pellico, Ilaria Magi, Antonina Minniti, Giuseppe Armentaro, Silvia Cavalli, Manuel Sette, Laura Giudice, Cristina Bochicchio, Alessandra Della Rossa, Antonio G Tavoni, Fabio Cacciapaglia, Stefano Stano, Martina Orlandi, Dilia Giuggioli, Marta Mosca, Roberto Caporali, Nicoletta Del Papa","doi":"10.1093/rheumatology/keaf292","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf292","url":null,"abstract":"<p><strong>Objectives: </strong>Digital ulcers (DUs) affect ∼50% of systemic sclerosis (SSc) patients, causing significant pain and functional impairment. Current management involves both systemic and local therapies. However, the burden in terms of pain and quality of life due to refractory DUs still remains heavy. While selexipag is approved for SSc-associated pulmonary arterial hypertension, its potential in treating DUs is unexplored. We aimed to evaluate the long-term efficacy of selexipag compared with iloprost in treating DUs.</p><p><strong>Methods: </strong>In this multicentre case-control study, we retrospectively evaluated 96 SSc patients with refractory DUs (32 treated with selexipag, 64 with iloprost), matched for gender, disease subset, and age. DU number, ischemic pain and Raynaud phenomenon (RP) severity were assessed at baseline, 6, 12, and 24 months. Pain and RP were evaluated using the Likert Pain Scale (LPS) and Raynaud Condition Score (RCS), respectively. Additionally, DUs recurrence and new onset were recorded. Healing rates were estimated using Kaplan-Meier analysis.</p><p><strong>Results: </strong>Selexipag showed higher efficacy with 87% of DUs healing rate vs 28% for iloprost at 96 weeks (p< 0.001). DUs number, RCS, and LPS showed significant improvement in selexipag-treated patients compared with iloprost (p< 0.001 for all) throughout 24 months. Selexipag-treated patients achieved faster healing (75% by week 40) and maintained significantly lower relapse rates (5% vs 45% at 24 months, p< 0.001). New DUs formation remained consistently lower in the selexipag group compared with the iloprost group (5% vs 40% at 24 months, p< 0.001).</p><p><strong>Conclusions: </strong>This observational study suggests that selexipag may be strongly effective in treating DUs refractory to conventional drugs.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209395","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":"Predictive factors and clinical outcomes of progressive pulmonary fibrosis in anti-threonyl (PL7) positive anti-synthetase syndrome.","authors":"Xueyan Shan, Zhenguo Huang, Guochun Wang, Yongpeng Ge","doi":"10.1093/rheumatology/keaf306","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf306","url":null,"abstract":"<p><strong>Objectives: </strong>Patients with anti-threonyl (PL7) positive anti-synthetase syndrome (ASS) exhibit a high prevalence of interstitial lung disease (ILD), which can progress to progressive pulmonary fibrosis (PPF) and increased mortality. This study aims to explore the clinical characteristics, imaging features, and predictive factors for PPF.</p><p><strong>Methods: </strong>A retrospective cohort of PL7-ASS patients at a single tertiary centre between January 2018 and December 2022, was analysed. Clinical, serological, and radiological data were collected at baseline and during follow-up.</p><p><strong>Results: </strong>The study included 69 PL7-ASS patients with ILD (mean age: 54.5 years; 73.9% female). Baseline CT imaging revealed the following patterns: cellular nonspecific interstitial pneumonia (cNSIP, 29%), fibrotic NSIP (fNSIP, 18.8%), organizing pneumonia (OP, 14.5%), OP-cNSIP overlap (15.9%), OP-fNSIP overlap (14.5%), and usual interstitial pneumonia (UIP, 7.2%). PPF developed in 39.1% of patients. Elevated levels of lactate dehydrogenase (LDH; HR = 1.021, 95% CI: 1.002-1.04, p= 0.031) and carbohydrate antigen 125 (CA125; HR = 1.164, 95% CI: 1.023-1.326, p= 0.022) were identified as independent predictors of PPF. Patients with UIP or OP-NSIP overlap patterns exhibited worse survival (p= 0.0479) and higher PPF prevalence (p= 0.029). Patients who developed PPF had significantly lower survival rates compared with those without PPF (HR = 5.120, 95% CI: 1.566-16.740, p= 0.018).</p><p><strong>Conclusion: </strong>PL7-ASS patients with ILD are at significant risk of developing PPF, which is associated with poor survival outcomes. Elevated LDH and CA125 levels may serve as reliable predictors of PPF, highlighting importance of early identification and aggressive management strategies.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209399","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":"Impact of immune checkpoint TIGIT on the activation and function of natural killer cells in rheumatoid arthritis patients","authors":"Ming Zhao, Rui Ma, Changsheng Xia, Jinghong Feng, Wenyi Li, Yan Long","doi":"10.1093/rheumatology/keaf305","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf305","url":null,"abstract":"Objectives This study aims to investigate the changes of circulating natural killer (NK) cells and their TIGIT expression diversities, and further explore the impact of TIGIT expression on the activity and function of NK cells in rheumatoid arthritis (RA) patients. Methods We comparatively assessed and compared the changes of NK cells, as well as their TIGIT expressions, among fifty-three cases of RA patients, twenty-three cases of healthy controls (HCs) and eleven osteoarthritis (OA) patients using flow cytometry and in-vitro cultures. CD25 and CD69 expression levels were used to evaluate the activation of NK subsets, and CD107α and GZMB expressions levels, IFN-γ production abilities were detected to indicate the functions of NK subsets. Results Circulating CD56brightNK cell levels were significantly elevated, while NKT cell levels were notably reduced in RA patients. In addition, NK cells manifested more activated phenotypes and enhanced functions in RA. Meanwhile, TIGIT expression on NK cells and subsets was significantly reduced, with an imbalanced TIGIT/CD226 ratio. TIGIT-expressing NK subsets showed lower CD25 expression levels and weakened IFN-γ production abilities in RA patients. Furthermore, in-vitro blockade of TIGIT-pathway with anti-TIGIT antibody enhanced NK cell activity and functions while activation of TIGIT-pathway with TIGIT-Fc chimera protein down-regulated NK cells activity and function. Conclusion Our results showed that decreased TIGIT expressions in RA patients and the TIGIT signalling pathway may participate in the activation and function of NK subsets, which show new insight for the exploration of RA pathogenesis.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":"17 1","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144218959","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":"Predicting trajectories of lung function decline in systemic sclerosis related interstitial lung disease.","authors":"Boyang Zheng, Mandana Nikpour, Wendy Stevens, Susanna Proudman, Kathleen Morrisroe, Mianbo Wang, Ada Man, Murray BaronP","doi":"10.1093/rheumatology/keaf270","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf270","url":null,"abstract":"<p><strong>Objective: </strong>Systemic sclerosis related interstitial lung disease (SSc-ILD) is a major cause of morbidity. We aimed to identify patients following similar trajectories of forced vital capacity (FVC) decline, examine their association with mortality and risk factors for FVC decline.</p><p><strong>Methods: </strong>This is a multicentre retrospective study of 444 SSc patients with ILD and ≤7-year disease duration. Patients were grouped based on similar FVC decline trajectories using semi-parametric modeling with latent class analysis. Survival was compared between the worst FVC trajectory group and the others. Logistic regression models with backwards selection were applied to identify predictors of FVC trajectory using baseline disease features.</p><p><strong>Results: </strong>Four FVC trajectory groups were identified. The most progressive trajectory declined by -2.18% per year and the other 3 trajectory groups were stable or progressed slowly. The most progressive group had a higher mortality rate than those with a stable/slow FVC trajectory (hazard ratio 2.95, 95%CI 1.74, 4.98). Baseline FVC (p< 0.001) and CRP elevation (p= 0.039) were associated the progressive trajectory. Baseline FVC ≤ 72% predicted the progressive trajectory with a sensitivity of 0.88 and specificity of 0.91. A lower baseline FVC was in turn associated with older age, Caucasian race, longer disease duration, ATA presence, and elevated CRP on exploratory analyses.</p><p><strong>Conclusion: </strong>Distinct FVC trajectories are associated with different survival outcomes and the most important predictor of a progressive FVC trajectory was existing ILD severity. More work is needed to assess the utility of imaging or paraclinical findings that can improve prediction of distinct FVC trajectories.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209398","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":"Safety of biologic therapy in kidney and liver transplant recipients with systemic inflammatory diseases: a real-world study from Israel.","authors":"Victoria Furer, Omer Kersh, Mark Berman, Ayelet Grupper, Liane Rabinowich, Hagit Peleg, Elisheva Pokroy-Shapira, Ori Elkayam","doi":"10.1093/rheumatology/keaf303","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf303","url":null,"abstract":"<p><strong>Objectives: </strong>Safety is a concern for solid-organ transplant (SOT) recipients with systemic inflammatory diseases (SID) treated with biologic therapy. This study evaluated the safety of biologic therapy in SOT recipients with SID.</p><p><strong>Methods: </strong>This retrospective study between 2000-2024 included 20 biologic-treated SOT recipients with SID matched to 56 SOT recipients controls without SID not treated with biologic therapy. The study compared post-transplant safety outcomes, with serious infections defined as the primary outcome. Kaplan-Meier survival analysis evaluated time-to-safety event outcomes.</p><p><strong>Results: </strong>The biologic-treated group included patients with mainly inflammatory bowel disease and Familial Mediterranean Fever treated with TNF and IL-1 inhibitors, respectively, with 60% (n = 12) treated with biologics over 5 years post-SOT. There was a non-significant trend for serious infections in the biologic-treated group vs controls, 40% (n = 8) vs 23.21% (n = 13), p= 0.15, with urinary tract infection being the most prevalent in both groups. Recurrent serious infections were more prevalent in the biologic-treated group vs controls, 20% (n = 4) vs 12.5% (n = 2), p= 0.029. No opportunistic infections were observed. No graft rejection occurred in the biologic-treated group compared with 5.37% in controls. Cancer rates were comparable in the biologic-treated group vs controls, 15% vs 7.14%, p= 0.3. There were 2 deaths attributed to COVID-19 infection and a car accident compared with none in the control group, p= 0.003. There were 6 cases of biologic treatment discontinuation, mainly due to loss of efficacy.</p><p><strong>Conclusion: </strong>This real-world cohort based on 5-year follow-up since the post-SOT biologic initiation supports the feasibility of biologic therapy in SOT recipients with SID.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209400","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":"Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study.","authors":"Vincent Sobanski, Jeska de Vries-Bouwstra, Anna-Maria Hoffmann-Vold, Dörte Huscher, Margarida Alves, Marco Matucci-Cerinic, Gabriela Riemekasten, Mengtao Li, László Czirják, Otylia Kowal-Bielecka, Yannick Allanore, Nils Schoof, Oliver Distler","doi":"10.1093/rheumatology/keaf264","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf264","url":null,"abstract":"<p><strong>Objectives: </strong>This study assessed how changes in lung function, skin fibrosis and digital ulceration (DU) burden predict mortality in patients with SSc-associated interstitial lung disease (SSc-ILD), the leading cause of death in SSc.</p><p><strong>Methods: </strong>Adult SSc-ILD patients from the European Scleroderma Trials and Research (EUSTAR) database enrolled since January 2009 with a date of diagnosis, a follow-up visit for change evaluation within 12 months plus a further visit, or mortality information were eligible. Twelve-month changes in lung function (per cent predicted forced vital capacity [FVC%pred] and diffusing capacity of the lungs for carbon monoxide [DLCO%pred]), modified Rodnan skin score (mRSS) and change in DU burden were assessed for associations with survival, using multivariable Cox regression analyses adjusted for age, sex, smoking status and immunosuppressive therapy.</p><p><strong>Results: </strong>Of 893 SSc-ILD patients included, 94 (10.5%) died over a mean follow-up of 39.0 ± 23.9 months. Absolute deterioration in FVC >10%pred within 12 months (n = 78/638 evaluable) was predictive for decreased survival (hazard ratio [HR] 3.81; 95% CI 1.67-8.66), as were composite measures combining (i) >10% FVC decline or mRSS worsening (HR 2.82; 95% CI 1.43-5.56) and (ii) FVC decline ≥10% or 5-9% with DLCO decline ≥15% (HR 3.42; 95% CI 1.68-7.00), but not changes in DLCO, mRSS or DU burden alone.</p><p><strong>Conclusions: </strong>Changes in lung function and skin fibrosis within 12 months should be considered when evaluating risk of mortality. The effect of pharmacological treatments aiming at stabilization of these variables should be evaluated prospectively in clinical trials.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209396","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":"Measuring what matters! the role of AAV-PRO in patient-centered care for EGPA: Insights from the study by Delvino et al.","authors":"Emre Bilgin","doi":"10.1093/rheumatology/keaf302","DOIUrl":"https://doi.org/10.1093/rheumatology/keaf302","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209397","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}