{"title":"Susac syndrome: challenges of interpreting treatment data in a rare disease","authors":"Todd A Hardy","doi":"10.1016/S2665-9913(24)00267-4","DOIUrl":"10.1016/S2665-9913(24)00267-4","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e2-e3"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142298936","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}
Emanuel Della-Torre MD PhD , Rosaria Talarico MD PhD , Jose Ballarin MD , Emanuele Bozzalla-Cassione MD , Chiara Cardamone MD , Cosimo Cigolini MD , Francesco Ferro MD , Tomas Fonseca MD , Prof George E Fragoulis MD , Ilaria Galetti Ing , Maria Gerosa MD , José Hernández-Rodríguez MD , Marco Lanzillotta MD , Diana Marinello , Prof Thierry Martin MD , Prof Fernando Martinez-Valle MD , Maria Maślińska MD , Michele Moretti MD , Prof Marta Mosca MD , Prof Ulf Müller-Ladner MD , Tobias Alexander MD
{"title":"Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework","authors":"Emanuel Della-Torre MD PhD , Rosaria Talarico MD PhD , Jose Ballarin MD , Emanuele Bozzalla-Cassione MD , Chiara Cardamone MD , Cosimo Cigolini MD , Francesco Ferro MD , Tomas Fonseca MD , Prof George E Fragoulis MD , Ilaria Galetti Ing , Maria Gerosa MD , José Hernández-Rodríguez MD , Marco Lanzillotta MD , Diana Marinello , Prof Thierry Martin MD , Prof Fernando Martinez-Valle MD , Maria Maślińska MD , Michele Moretti MD , Prof Marta Mosca MD , Prof Ulf Müller-Ladner MD , Tobias Alexander MD","doi":"10.1016/S2665-9913(24)00192-9","DOIUrl":"10.1016/S2665-9913(24)00192-9","url":null,"abstract":"<div><div>IgG4-related disease is a rare fibroinflammatory condition. Prompt recognition is fundamental to initiate treatment and to prevent organ damage. Diagnostic and classification criteria are primarily intended for use by clinicians with established expertise in IgG4-related disease. Absence of disease awareness among primary care physicians and specialists without expertise in IgG4-related disease remains the main cause of diagnostic delay. We aimed to identify red flags that might increase the suspicion of IgG4-related disease in primary and secondary care settings. A task force of experts in IgG4-related disease from the European Reference Network for Rare Connective Tissue Diseases (ERN-ReCONNET), patient representatives, and primary care physicians derived potential red flags for IgG4-related disease through a systematic literature search and a level of agreement exercise. Five red flags reached 100% agreement among experts: swelling in one or more organ system; pancreatic and biliary tree involvement; increased serum IgG4; IgG4<sup>+</sup> plasma cell tissue infiltration; and obliterative phlebitis. Red flags for IgG4-related disease are intended for use in primary and secondary care to improve referral to centres of expertise and prompt early diagnosis.</div></div>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e64-e71"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564656","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}
Merlijn Wind , Juan J Fierro , Kitty W M Bloemenkamp , Karina de Leeuw , A Titia Lely , Maarten Limper , Marieke Sueters , Y K Onno Teng , Isabel J Walter , Judith Kooiman
{"title":"Predictors of pregnancy outcomes in SLE – Authors' reply","authors":"Merlijn Wind , Juan J Fierro , Kitty W M Bloemenkamp , Karina de Leeuw , A Titia Lely , Maarten Limper , Marieke Sueters , Y K Onno Teng , Isabel J Walter , Judith Kooiman","doi":"10.1016/S2665-9913(24)00347-3","DOIUrl":"10.1016/S2665-9913(24)00347-3","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Page e13"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792624","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}
Sherry Yang BS , Leah Santacroce MS , Jamie E Collins PhD , Candace H Feldman MD ScD
{"title":"Association of historical redlining and present-day racialised economic segregation with health-care utilisation among individuals with rheumatic conditions in Massachusetts and surrounding areas of the USA: a retrospective cohort study","authors":"Sherry Yang BS , Leah Santacroce MS , Jamie E Collins PhD , Candace H Feldman MD ScD","doi":"10.1016/S2665-9913(24)00235-2","DOIUrl":"10.1016/S2665-9913(24)00235-2","url":null,"abstract":"<div><h3>Background</h3><div>Structural racism lies at the root of inequities; however its impact on rheumatology care is understudied. Redlining was a US federal government-sponsored practice that mapped areas with high concentrations of Black and immigrant residents as hazardous for investment. We aimed to investigate the association of historical redlining and present-day racialised economic segregation, on health-care utilisation among individuals with rheumatic conditions in the US state of Massachusetts and surrounding areas.</div></div><div><h3>Methods</h3><div>This retrospective observational cohort study used multihospital data from the Mass General Brigham Research Patient Data Registry to identify individuals aged ≥ 18 years living in Massachusetts and surrounding areas, with two or more International Classification of Diseases codes for a rheumatic condition. Individuals were included if they received care between Jan 1, 2000, and May 1, 2023, at rheumatology practices affiliated with Mass General Brigham (Boston, MA, USA). Addresses were geocoded and overlaid with 1930s Home Owners' Loan Corporation (HOLC) redlining files. The Index of Concentration at the Extremes (ICE) for combined racial and income polarisation was constructed from US Census data. We used multilevel, multinomial logistic regression models to examine the odds of health-care utilisation separately by historical HOLC grade (A [best] to D [hazardous]) and ICE quintile (most deprived [1] to most privileged [5] race and income), adjusting for demographics, insurance, and comorbidities. People with lived experience of a rheumatic condition were not involved in the design or implementation of this study.</div></div><div><h3>Findings</h3><div>The cohort comprised 5597 individuals; 3944 (70·5%) of 5597 patients were female, 1653 (29·5%) were male, 657 (11·7%) were Black, 224 (4·0%) were Hispanic, and the median age was 63 (50–73) years. 1295 (23·1%) of 5597 individuals lived in the most historically redlined areas (HOLC D) and 1780 (31·8%) lived in areas with the most concentrated present-day racialised economic deprivation (ICE quintile 1). Individuals in historically redlined areas (HOLC D) had greater odds of having four or more missed appointments (odds ratio [OR] 1·78 [95% CI 1·21–2·61]; p=0·0033) and of three or more emergency department visits (2·69 [1·48–4·89]; p=0·0011) compared with those in the most desirable neighbourhoods (HOLC A). Individuals in areas with highly concentrated racial and economic deprivation (ICE quintile 1) had greater odds of four or more missed appointments (OR 2·11 [95% CI 1·65–2·71]; p<0·0001) and of three or more emergency department visits (2·97 [2·02–4·35]; p<0·0001) versus those in areas with highly concentrated privilege (ICE quintile 5).</div></div><div><h3>Interpretation</h3><div>Historical redlining could be a structural determinant of inequities in present-day health-care utilisation patterns. Policy interventions that disma","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 1","pages":"Pages e33-e43"},"PeriodicalIF":15.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142693678","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":"Heading for remission and its continuation in rheumatoid arthritis","authors":"Paul Studenic","doi":"10.1016/S2665-9913(24)00336-9","DOIUrl":"10.1016/S2665-9913(24)00336-9","url":null,"abstract":"","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 4","pages":"Pages e222-e224"},"PeriodicalIF":15.0,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142883382","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}
Judith W Heutz MD , Pascal H P de Jong MD PhD , Marloes Verstappen MD PhD , Prof Annette H M van der Helm-van Mil MD PhD , Elise van Mulligen PhD
{"title":"Sustained DMARD-free remission in subgroups of patients with rheumatoid arthritis: an analysis of two prospective cohorts with early arthritis","authors":"Judith W Heutz MD , Pascal H P de Jong MD PhD , Marloes Verstappen MD PhD , Prof Annette H M van der Helm-van Mil MD PhD , Elise van Mulligen PhD","doi":"10.1016/S2665-9913(24)00234-0","DOIUrl":"10.1016/S2665-9913(24)00234-0","url":null,"abstract":"<div><h3>Background</h3><div>About 20% of patients with rheumatoid arthritis on disease-modifying antirheumatic drugs (DMARDs) can reach sustained DMARD-free remission. Nonetheless, the 2022 EULAR recommendations discourage complete cessation of DMARDs due to flare risk. The evidence behind this recommendation is obtained from trial populations using biological DMARDs, representing only a subgroup of the total population of patients with rheumatoid arthritis. We hypothesised that patients requiring biological DMARDs represent a subgroup that is less capable of reaching sustained DMARD-free remission compared with patients not requiring a biological DMARD.</div></div><div><h3>Methods</h3><div>In this study we used data from two prospectively followed up populations of patients with early rheumatoid arthritis, the Leiden Early Arthritis Clinic (EAC) and the treatment in the Rotterdam Early Arthritis Cohort (tREACH), a treat-to-target steered trial in which biological DMARDs were started when patients had inadequate response to triple DMARD-therapy (methotrexate, sulfasalazine, and hydroxychloroquine). Patient partners were involved in the design of both the EAC and tREACH. The primary outcome was sustained DMARD-free remission, which was defined as absence of clinical synovitis after discontinuation of DMARDs for at least 1 year. Patients who did or did not receive biological DMARDs in 5 years (EAC) or 3 years (tREACH) were compared using Kaplan–Meier curves.</div></div><div><h3>Findings</h3><div>627 patients from the EAC were included, of whom 391 (62%) were female and 236 (38%) were male. The mean age was 60 years (SD 14) and 502 (95%) of 529 patients were White. 89 (14%) of 627 patients had ever used a biological DMARD and 538 (86%) had never used a biological DMARD. None of the patients that used a biological DMARD reached sustained DMARD-free remission, whereas 37% of the patients who never used a biological DMARD reached sustained DMARD-free remission at 5 years (hazard ratio [HR] 0·02, 95% CI 0·00–0·10; p<0·0001). From the tREACH population, 425 patients were included in the study. 286 (67%) patients were female, 139 (33%) were male, and the mean age was 54 years (SD 14); ethnicity data not recorded. 154 (36%) of 425 patients had ever used a biological DMARD and 271 (64%) had never used a biological DMARD during follow-up. None of the patients that used a biological DMARD reached sustained DMARD-free remission, whereas 15% of patients who never used a biological DMARD reached sustained DMARD-free remission at 3 years (HR 0·03, 95% CI 0·00–0·21; p<0·0001).</div></div><div><h3>Interpretation</h3><div>For the subgroup of patients with rheumatoid arthritis who require biological DMARDs, sustained DMARD-free remission does not seem attainable. In contrast, in patients with rheumatoid arthritis who do not require biological DMARDs, DMARD-free remission is attainable. These data suggest that the current EULAR recommendation to not stop DMARD ","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":"7 4","pages":"Pages e252-e260"},"PeriodicalIF":15.0,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142883388","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}