Lancet Rheumatology最新文献

{"title":"Predicting the risk of subsequent progression in patients with systemic sclerosis-associated interstitial lung disease with progression: a multicentre observational cohort study.","authors":"Anna-Maria Hoffmann-Vold, Liubov Petelytska, Håvard Fretheim, Trond Mogens Aaløkken, Mike Oliver Becker, Hilde Jenssen Bjørkekjær, Cathrine Brunborg, Cosimo Bruni, Christian Clarenbach, Phuong Phuong Diep, Rucsandra Dobrota, Michael T Durheim, Muriel Elhai, Thomas Frauenfelder, Suiyuan Huang, Suzana Jordan, Emily Langballe, Øyvind Midtvedt, Carina Mihai, Erica Mulcaire-Jones, Janelle Vu Pugashetti, Marco Sprecher, Justin Oldham, Øyvind Molberg, Dinesh Khanna, Oliver Distler","doi":"10.1016/S2665-9913(25)00026-8","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00026-8","url":null,"abstract":"<p><strong>Background: </strong>In patients with systemic sclerosis, it is common practice to treat interstitial lung disease (ILD) in patients in whom progression has already occurred. We sought to clarify whether observed progression of systemic sclerosis-associated ILD confers risk for subsequent progression.</p><p><strong>Methods: </strong>In this multicentre observational cohort study, based on an analysis of prospectively collected data, we included patients with systemic sclerosis-associated ILD aged 18 years or older at diagnosis, who fulfilled the 2013 American College of Rheumatology-European Association of Alliances in Rheumatology systemic sclerosis classification criteria. The main cohort (diagnosed between January 2001 and December 2019) was consecutively followed up annually over 4 years at the Department of Rheumatology at the Oslo University Hospital, Norway, and the Department of Rheumatology at the University Hospital Zurich, Switzerland. We applied four definitions of ILD progression: the primary definition was forced vital capacity (FVC) decline of 5% or more, and secondary definitions included FVC decline of 10% or more, progressive pulmonary fibrosis (PPF), and progressive fibrosing ILD (PF-ILD). We applied these definitions at each annual visit after the first (visit 1). We validated our findings in an enriched cohort that included patients from the main cohort with systemic sclerosis-associated ILD and short disease duration of less than 3 years along with patients diagnosed between January 2003 and September 2019 from the Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. Multivariable logistic regression analyses were applied to predict ILD progression and its effect on mortality. There was no involvement of people with lived experience in this study.</p><p><strong>Findings: </strong>Of 231 patients with systemic sclerosis-associated ILD from the main cohort (mean age 48·0 years [SD 14·6], 176 [76%] female and 55 [24%] male), 71 (31%) had ILD progression as defined by an FVC decline of 5% or more between visit 1 and visit 2, 38 (16%) as defined by an FVC decline of 10% or more, 39 (17%) as defined by PPF, and 89 (39%) defined by PF-ILD. In multivariable logistic regression analyses, adjusted for risk factors for progressive systemic sclerosis-associated ILD and immunosuppressive treatment, we found that ILD progression, defined by FVC decline of 5% or more, from visit 1 to visit 2 reduced the risk for further progression from visit 2 to visit 3 (odds ratio [OR] 0·28 [95% CI 0·12-0·63]; p=0·002) and that there was no risk for subsequent progression using the other definitions (FVC decline of ≥10%: 0·57 [0·16-1·99; p=0·38]; PPF: 0·93 [0·39-2·22; p=0·88]; and PF-ILD: 0·69 [0·35-1·36]; p=0·28]). Using the primary definition of progression, we found the same results in the enriched systemic sclerosis-associated ILD cohort, wherein 41 (34%) of 121 patients had progression defined by an FVC decline of 5% or ","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144095802","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Why is progressive ILD in systemic sclerosis so difficult to predict?","authors":"Kathleen Morrisroe, Murray Barron","doi":"10.1016/S2665-9913(25)00068-2","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00068-2","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144095805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The use of IL-1 and IL-6 inhibitors should be prioritised in Still's disease - Authors' reply.","authors":"Yujie Shen, Jinchao Jia, Jialin Teng, Chengde Yang, Qiongyi Hu","doi":"10.1016/S2665-9913(25)00110-9","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00110-9","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144086886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The use of IL-1 and IL-6 inhibitors should be prioritised in Still's disease.","authors":"Stéphane Mitrovic, Rashmi Sinha, Fabrizio De Benedetti, Bruno Fautrel","doi":"10.1016/S2665-9913(25)00109-2","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00109-2","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144086889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vascular Ehlers-Danlos syndrome: a heritable condition with potentially fatal consequences.","authors":"Emily Woods, Jessica M Bowen, Diana S Johnson, Glenda J Sobey","doi":"10.1016/S2665-9913(25)00128-6","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00128-6","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144086892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term effect of anifrolumab on patient-reported outcomes in systemic lupus erythematosus (TULIP-LTE): a randomised, placebo-controlled, phase 3 long-term extension trial.","authors":"Vibeke Strand, Kenneth C Kalunian, Kai Wai Lee, Caroline Seo, Gabriel Abreu, Raj Tummala, Hussein Al-Mossawi, Elizabeth A Duncan, Catharina Lindholm","doi":"10.1016/S2665-9913(25)00022-0","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00022-0","url":null,"abstract":"<p><strong>Background: </strong>In the TULIP-1 and TULIP-2 phase 3 trials, anifrolumab treatment was associated with clinical efficacy and clinically meaningful improvements in multiple patient-reported outcomes over 52 weeks in patients with moderate-to-severe systemic lupus erythematosus (SLE). At the end of TULIP-1 and TULIP-2 (week 52), eligible patients could reconsent to enter a 3-year long-term extension trial (TULIP-LTE). Here, we investigated changes in patient-reported outcomes during treatment with anifrolumab or placebo for up to 4 years in patients with SLE who were receiving standard therapy.</p><p><strong>Methods: </strong>TULIP-LTE was a 3-year randomised, double-blind, placebo-controlled, phase 3 long-term extension of the 1-year TULIP-1 and TULIP-2 trials. Patients who were randomly assigned to receive anifrolumab 300 mg (n=257) or placebo (n=112) every 4 weeks in the TULIP-1 and TULIP-2 trials and who continued the same treatment in TULIP-LTE were assessed. Exploratory endpoints were changes from baseline in patient-reported outcomes (Short Form-36 version 2 acute recall [SF-36v2 (acute)], Patient Global Assessment of disease activity, EuroQoL 5 Dimensions-5 Levels [EQ-5D-5L], and Work Productivity and Activity Impairment-Lupus) and health utility indices, Short-Form 6-Dimension (SF-6D), derived from SF-36v2 (acute), and EQ-5D-5L. All analyses were done in the modified intention-to-treat population, which included all randomly assigned patients from TULIP-1 and TULIP-2 who received at least one dose of the same treatment during the long-term extension phase. Least-squares mean patient-reported outcome scores were adjusted using a repeated measures model. No people with lived experience were directly involved in the study design or implementation. This trial was registered with ClinicalTrials.gov, NCT02794285.</p><p><strong>Findings: </strong>Between June 30, 2016, and Oct 12, 2018, 369 patients entered the long-term extension study and were included in this exploratory analysis (257 in the anifrolumab group and 112 in the placebo group). Mean age of patients was 42·8 years (SD 11·5), 340 (92%) of 369 patients were women and 29 (8%) were men, 250 (68%) patients were White and 82 (22%) self-identified as Hispanic or Latino. Patient-reported outcome scores improved from baseline in both groups during the 4-year treatment period. At week 208, improvement from baseline in SF-36v2 (acute) was numerically higher in the anifrolumab group than the placebo group for the bodily pain domain (least-squares mean difference 5·9 [95% CI -0·7 to 12·5]) and the mental health domain (3·7 [-1·2 to 8·6]). Improvements from baseline in SF-6D were generally greater in the anifrolumab group than the placebo group, with numerical differences between groups evident from as early as week 24 (least-squares mean difference 0·013 [-0·007 to 0·032]) and maintained at week 208 (0·016 [-0·010 to 0·042]).</p><p><strong>Interpretation: </strong>Over 4 years of t","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144054713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patient-reported outcomes: a new basis for prediction models?","authors":"Tanja Stamm, Preston Long, Valentin Ritschl, Erika Mosor, Peter Mandl","doi":"10.1016/S2665-9913(25)00066-9","DOIUrl":"https://doi.org/10.1016/S2665-9913(25)00066-9","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":" ","pages":""},"PeriodicalIF":15.0,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Local immune effector cell-associated toxicity syndrome in CAR T-cell treated patients with autoimmune disease: an observational study","authors":"Melanie Hagen MD ,&nbsp;Prof Fabian Müller MD ,&nbsp;Andreas Wirsching MD ,&nbsp;Soraya Kharboutli MD ,&nbsp;Prof Silvia Spoerl MD ,&nbsp;Christina Düsing MD ,&nbsp;Tobias Krickau MD ,&nbsp;Prof Markus Metzler MD ,&nbsp;Simon Völkl PhD ,&nbsp;Michael Aigner MD ,&nbsp;Sascha Kretschmann PhD ,&nbsp;Ingrid Vasova MD ,&nbsp;Prof Marc Saake MD ,&nbsp;Stefan Schliep MD ,&nbsp;Torsten Kubacki MD ,&nbsp;Prof Nicolas Hunzelmann MD ,&nbsp;Laura Bucci MD ,&nbsp;Jule Taubmann MD ,&nbsp;Christina Bergmann MD ,&nbsp;Andrea-Hermina Györfi MD ,&nbsp;Prof Georg Schett MD","doi":"10.1016/S2665-9913(25)00091-8","DOIUrl":"10.1016/S2665-9913(25)00091-8","url":null,"abstract":"<div><h3>Background</h3><div>CD19-targeting chimeric antigen receptor (CAR) T-cell therapy has advanced treatment strategies for severe autoimmune diseases such as systemic lupus erythematosus (SLE), systemic sclerosis, and idiopathic inflammatory myopathy. Data regarding side-effects are mostly generated from patients with malignancies, but little is known about autoimmune disease-specific adverse events. This study aimed to describe autoimmune disease-specific adverse events that occur with CAR T-cell therapy.</div></div><div><h3>Methods</h3><div>In this observational study, patients of any age with autoimmune disease receiving CD19-targeting CAR T-cell therapy in two centres in Germany with a follow-up of at least 30 days were assessed for local organ-specific reactions occurring after CAR T-cell infusion. Observed reactions were documented according to localisation, time of onset, and duration, and were graded for severity (grade 1: spontaneous resolution; grade 2: glucocorticoid treatment due to symptoms lasting &gt;1 week or presence of relevant inner organ involvement; grade 3: prolonged or new hospitalisation; grade 4: intensive care treatment). People with related lived experience were involved in the study design and implementation.</div></div><div><h3>Findings</h3><div>Between March 1, 2021, and Oct 31, 2024, 39 patients with autoimmune disease were treated with CD19-targeting CAR T cells (20 with SLE, 13 with systemic sclerosis, six with idiopathic inflammatory myopathy). 25 (64%) patients were female and 14 (36%) were male. Median age was 36 years (IQR 22–44). 54 local reactions, which we termed local immune effector cell-associated toxicity syndrome (LICATS), were recorded, affecting 30 (77%) patients with a median time of onset of 10 days (IQR 9–21) from CAR T-cell infusion and a median duration of 11 days (5–14). LICATS exclusively occurred during the B-cell aplasia phase and only involved organs previously affected by the respective autoimmune disease. The most frequently affected organs were the skin (19 [35%] of 54) and the kidneys (12 [22%]). Most cases of LICATS were mild (grade 1: 35 [65%]; grade 2: 16 [30%]). Only three cases were grade 3. All events of LICATS resolved without sequelae.</div></div><div><h3>Interpretation</h3><div>LICATS is a new form of toxicity in patients with autoimmune disease receiving CD19-targeting CAR T-cell therapy, most likely based on the cleansing of immune cells from the affected organs. It is self-limited, organ-specific, and usually mild in its intensity.</div></div><div><h3>Funding</h3><div>Deutsche Forschungsgemeinschaft (DFG), German Cancer Aid, Bundesministerium für Bildung und Forschung, European Union, Staedtler Foundation, Lupus Research Alliance, and donations from the Bendel family and the Bleyl family.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 6","pages":"Pages e424-e433"},"PeriodicalIF":15.0,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144006991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A new toxicity syndrome in patients with autoimmune disease treated with CAR T-cell therapy","authors":"Samuel D Good ,&nbsp;Elizabeth R Volkmann","doi":"10.1016/S2665-9913(25)00100-6","DOIUrl":"10.1016/S2665-9913(25)00100-6","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 6","pages":"Pages e382-e384"},"PeriodicalIF":15.0,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144054859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Laura Coates: all about the people","authors":"Heather Van Epps","doi":"10.1016/S2665-9913(25)00107-9","DOIUrl":"10.1016/S2665-9913(25)00107-9","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 5","pages":"Page e313"},"PeriodicalIF":15.0,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143873444","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}