Vandana Suresh , Mary K. Crow , Jennifer H. Anolik , S. Louis Bridges Jr. , David S. Pisetsky
{"title":"Highlights from the 2024 Rheumatoid Arthritis Research Summit","authors":"Vandana Suresh , Mary K. Crow , Jennifer H. Anolik , S. Louis Bridges Jr. , David S. Pisetsky","doi":"10.1016/j.semarthrit.2024.152589","DOIUrl":"10.1016/j.semarthrit.2024.152589","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152589"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142676541","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":"Immune checkpoint inhibitors and rheumatoid arthritis: All roads lead to PD-1?","authors":"Laura C. Cappelli","doi":"10.1016/j.semarthrit.2024.152582","DOIUrl":"10.1016/j.semarthrit.2024.152582","url":null,"abstract":"<div><div>Immune checkpoint molecules like PD-1 and its ligand PD-L1 and CTLA-4 are important regulators of the immune system. Medications blocking these pathways, immune checkpoint inhibitors, have been used to treat a variety of malignancies, while drugs agonizing these pathways, like abatacept, have been used in treating autoimmune diseases. Modulation of the PD-1/PD-L1 axis has become important for rheumatologists to understand in several different clinical scenarios. Currently, PD-1 agonists are being developed for treatment of rheumatoid arthritis (RA). In addition to patients with RA being potentially treated with PD-1 agonists, patients with rheumatoid arthritis may be treated with anti-PD-1/PD-L1 immune checkpoint inhibitors if they develop cancer. Finally, patients treated with immune checkpoint inhibitors may develop de novo inflammatory arthritis and be referred to rheumatology for management. In all three scenarios, there remain many unanswered clinical and translational questions. The parallel development of therapeutics antagonizing and agonizing the PD-1/PD-L1 pathway presents a unique chance for discovery in inflammatory arthritis.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152582"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142693552","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}
Marco Binda , Augusta Ortolan , Beatrice Moccaldi , Mariangela Salvato , Anna Cuberli , Roberto Padoan , Andrea Doria , Elisabetta Zanatta
{"title":"Phenotype of diffuse cutaneous systemic sclerosis patients with positive anticentromere antibodies: A systematic literature review and meta-analysis","authors":"Marco Binda , Augusta Ortolan , Beatrice Moccaldi , Mariangela Salvato , Anna Cuberli , Roberto Padoan , Andrea Doria , Elisabetta Zanatta","doi":"10.1016/j.semarthrit.2024.152606","DOIUrl":"10.1016/j.semarthrit.2024.152606","url":null,"abstract":"<div><h3>Objectives</h3><div>Anticentromere antibodies (ACA) are typically found in limited cutaneous systemic sclerosis (lcSSc), whereas patients with anti-topoisomerase I antibodies (ATA) usually exhibit diffuse cutaneous involvement (dcSSc). We aimed to investigate the clinical phenotype and outcome of ACA-dcSSc.</div></div><div><h3>Methods</h3><div>A systematic literature review was conducted (January 1970 to April 2023) across MEDLINE, Scopus and OVID, to define whether SSc patients (population) within the ACA-dcSSc subset (exposure) had higher/lower risk for major organ involvement (interstitial lung disease-ILD, pulmonary hypertension-PH, primary myocardial involvement-PMI, scleroderma renal crisis-SRC) and mortality (outcomes) compared to ACA-lcSSc and ATA-dcSSc. Inclusion criteria were: 1) adult SSc patients with identifiable demographic and clinical features by subtype; 2) observational studies. The quality of the studies was evaluated by the Newcastle–Ottawa Scale. Random-effects meta-analysis was performed to compare odds ratios (OR) for major organ involvement, and the 5- and 10-year mortality of ACA-dcSSc with the other subsets.</div></div><div><h3>Results</h3><div>Out of 1570 hits, six articles were included, identifying 177 ACA-dcSSc patients. In ACA-dcSSc, ILD was more frequent than in ACA-lcSSc (OR 2.60; 95 %CI 1.39–4.87) but less frequent compared to ATA-dcSSc (OR 0.17; 95 %CI 0.10–0.29). ACA-dcSSc patients had a higher prevalence of PH vs. both conventional subsets; PMI and SRC were more frequent in ACA-dcSSc compared to ACA-lcSSc, and similar to ATA-dcSSc. While 5-year survival rates were comparable among the subsets, ACA-dcSSc patients exhibited a lower 10-year mortality than ATA-dcSSc (OR 0.42; 95 %CI 0.2–0.85).</div></div><div><h3>Conclusion</h3><div>Although uncommon, the ACA-dcSSc subset appears to have a distinct clinical phenotype, with a better prognosis than ATA-dcSSc.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152606"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142807850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Melanie H. Smith , Zilong Bai , Amit Lakhanpal , Daniel Ramirez , Edward DiCarlo , Laura Donlin , Dana Orange , Susan M. Goodman
{"title":"Characterizing molecular targets in difficult-to-treat rheumatoid arthritis","authors":"Melanie H. Smith , Zilong Bai , Amit Lakhanpal , Daniel Ramirez , Edward DiCarlo , Laura Donlin , Dana Orange , Susan M. Goodman","doi":"10.1016/j.semarthrit.2024.152588","DOIUrl":"10.1016/j.semarthrit.2024.152588","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152588"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142740429","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}
Gustavo G.M. Balbi , Pedro Gaspar , Hannah Cohen , David A. Isenberg , Doruk Erkan , Danieli Andrade , APS ACTION Extended “Damage” Working Group
{"title":"Damage Index for Antiphospholipid Syndrome (DIAPS): An Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) “Damage” working group report on strengths and limitations","authors":"Gustavo G.M. Balbi , Pedro Gaspar , Hannah Cohen , David A. Isenberg , Doruk Erkan , Danieli Andrade , APS ACTION Extended “Damage” Working Group","doi":"10.1016/j.semarthrit.2024.152605","DOIUrl":"10.1016/j.semarthrit.2024.152605","url":null,"abstract":"<div><h3>Objectives</h3><div>To gather the perspectives of APS ACTION members regarding the strengths and limitations of Damage Index for Antiphospholipid Syndrome (DIAPS); and establish recommendations for the improvement of DIAPS.</div></div><div><h3>Methods</h3><div>APS ACTION members were invited to answer a survey regarding their satisfaction with DIAPS scoring system and individual items. The level of agreement (LoA) among members with the inclusion of individual items in DIAPS was calculated (LoA of <75% was considered disagreement). Respondents’ open-ended comments about DIAPS limitations were also collected, which helped formulate our recommendations for DIAPS improvement.</div></div><div><h3>Results</h3><div>Forty-two APS ACTION members (58.3%) answered the survey. Of them, 26 (61.9%) were satisfied, 4 (9.5%) were neutral, and 12 (28.6%) were dissatisfied with the current DIAPS scoring system. Fifteen items (39.5%) presented a LoA <75% regarding the inclusion in DIAPS. Respondents provided comments that were grouped under six main categories related to concerns about: a) definitions and attribution of damage (including causality and temporal relationship); b) scoring system; c) overlapping items; d) specific items (exclusion of redundant items and inclusion of additional ones); e) the need to incorporate multiple events; and f) feasibility and practicality. Finally, the APS ACTION “Damage” Working Group developed 7 recommendations that should be considered for the next generation DIAPS.</div></div><div><h3>Conclusion</h3><div>Approximately 60% of respondents were satisfied with DIAPS and its definitions; however, our survey demonstrated that there is substantial room to improve the current damage index for APS. Efforts for updating DIAPS should consider the APS ACTION “Damage” Working Group recommendations.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152605"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142814218","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}
Guy Katz, Claire E. Cook, Xiaoqing Fu, Andrew J. King, John H. Stone, Hyon K. Choi, Zachary S. Wallace
{"title":"Defining cause of death in a contemporary cohort with ANCA-associated vasculitis (AAV): A comparison of electronic health record and death certificate data","authors":"Guy Katz, Claire E. Cook, Xiaoqing Fu, Andrew J. King, John H. Stone, Hyon K. Choi, Zachary S. Wallace","doi":"10.1016/j.semarthrit.2024.152609","DOIUrl":"10.1016/j.semarthrit.2024.152609","url":null,"abstract":"<div><h3>Objectives</h3><div>Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) face excess mortality compared with the general population. Mortality in clinical epidemiology research is often examined using death certificate diagnosis codes; however, the sensitivity of such codes in AAV is unknown.</div></div><div><h3>Methods</h3><div>We performed a retrospective cohort study using the Mass General Brigham AAV Cohort, including patients with AAV who died between 2002 and 2019. Causes of death were determined by electronic health record (EHR) review (reference gold standard) and via cause of death diagnosis codes on death certificates. We calculated the sensitivity of death certificate diagnosis codes for AAV.</div></div><div><h3>Results</h3><div>Of 684 patients in the registry, 184 died, 92 (52 %) of whom had adequate EHR data available determine cause of death and 72 (40 %) of whom had both EHR and death certificate data available. Death due to AAV, infection, cardiovascular disease, and cancer occurred in 8 %, 29 %, 5 %, and 18 %, respectively, when ascertained by manual review, as opposed to 0 %, 11 %, 25 %, and 21 %, as determined by death certificates. The sensitivity of AAV diagnosis codes for AAV was 16.6 % (95 % CI: 10.5, 22.6) among all patients with death certificate data available.</div></div><div><h3>Conclusion</h3><div>In a contemporary cohort of patients with AAV, infection was the most common cause of death, while death due to AAV itself was rare. We found a high degree of discordance between causes of death determined by manual review and death certificate diagnosis codes. Mortality research on AAV should include linkage to medical records data to reduce potential bias.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152609"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903417","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":"Microbial pathways contributing to the pathogenesis of rheumatoid arthritis","authors":"Kristine A. Kuhn","doi":"10.1016/j.semarthrit.2024.152587","DOIUrl":"10.1016/j.semarthrit.2024.152587","url":null,"abstract":"<div><div>None.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152587"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142627716","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":"The cause is worse than the effect: Inequities in the United States health system; how could we change them?","authors":"Iris Navarro-Millán","doi":"10.1016/j.semarthrit.2024.152590","DOIUrl":"10.1016/j.semarthrit.2024.152590","url":null,"abstract":"<div><div>Abstract Para</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152590"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142639710","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}
Catherine Deffendall , Sarah Green , Ashley Suh , Nikol Nikolova , Katherine Walker , Raeann Whitney , Lee Wheless , Sarah Osmundson , April Barnado
{"title":"Peripartum maternal outcomes in individuals with systemic lupus erythematosus in a real-world electronic health record cohort","authors":"Catherine Deffendall , Sarah Green , Ashley Suh , Nikol Nikolova , Katherine Walker , Raeann Whitney , Lee Wheless , Sarah Osmundson , April Barnado","doi":"10.1016/j.semarthrit.2024.152603","DOIUrl":"10.1016/j.semarthrit.2024.152603","url":null,"abstract":"<div><h3>Objective</h3><div>Few studies have examined peripartum maternal outcomes in systemic lupus erythematosus (SLE). Using a de-identified electronic health record (EHR) cohort of individuals with and without SLE, we compared rates of peripartum maternal outcomes including maternal infections, blood transfusions, hospital length of stay, and SLE flares.</div></div><div><h3>Methods</h3><div>We identified deliveries among individuals with SLE and individuals without autoimmune disease using a previously validated algorithm. Peripartum maternal infection was assessed up to 6 weeks postpartum. Using Chi-square and Mann-Whitney U tests, we compared peripartum outcomes in SLE and control deliveries. We performed mixed effects models to estimate the association of SLE case status with peripartum outcomes. We assessed for SLE flares up to 6 months postpartum using chart review of rheumatology notes and the 2009 revised SELENA Flare Index. We evaluated SLE medications prescribed during pregnancy and at time of delivery on peripartum outcomes.</div></div><div><h3>Results</h3><div>We identified 185 deliveries to 142 individuals with SLE and 468 deliveries to 241 control individuals without autoimmune diseases. Mean length of hospital stay was longer for individuals with SLE compared to controls (3.1 ± 2.0 vs. 2.4 ± 1.0 days, <em>p</em> < 0.001). In a mixed effects model, peripartum infection was significantly associated with SLE case status (OR = 6.18, 95 % CI 2.73 – 13.98, <em>p</em> < 0.01), Cesarean section (OR = 5.00, 95 % CI 2.16 – 11.57, <em>p</em> < 0.01), and age at delivery (OR = 0.92, 95 % CI 0.86 - 0.99, <em>p</em> = 0.03) after adjusting for race. Transfusion was also significantly associated with SLE case status (OR = 9.05, 95 % CI 3.24–25.32, <em>p</em> < 0.01) and Black race (OR = 6.64, 95 % CI 1.47 – 30.02, <em>p</em> = 0.01) after adjusting for Cesarean section and age at delivery. We observed a postpartum flare rate of 32 % among individuals with SLE with 13 % characterized as mild, 41 % moderate, and 46 % severe. Antimalarial use in the postpartum period was associated with lower flare rate (43 % vs. 63 %, <em>p</em> = 0.04).</div></div><div><h3>Conclusions</h3><div>Individuals with SLE have increased rates of blood transfusions, longer hospital stays, and more frequent infections compared to control individuals in the peripartum period. We observed a postpartum flare rate of 32 %, and antimalarial use was associated with lower flare rate. Our findings demonstrate that the peripartum period remains a high-risk time for individuals with SLE with an ongoing need for close monitoring.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152603"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yongpeng Ge, Wei Jiang, Xin Lu, Guochun Wang , Xiaoming Shu
{"title":"Refractory cutaneous ulcers in anti-MDA5 dermatomyositis were not associated with rapidly progressive interstitial lung disease, and responded to tadalafil","authors":"Yongpeng Ge, Wei Jiang, Xin Lu, Guochun Wang , Xiaoming Shu","doi":"10.1016/j.semarthrit.2025.152651","DOIUrl":"10.1016/j.semarthrit.2025.152651","url":null,"abstract":"<div><h3>Objectives</h3><div>The purpose of this study was to analyze the characteristics of an ulcerative rash in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody and to assess the efficacy of tadalafil in the management of refractory cutaneous ulcers.</div></div><div><h3>Methods</h3><div>Medical records of DM patients with anti-MDA5 antibody were reviewed. Skin rash data, radiologic characteristic and serum markers were collected. Patients with refractory cutaneous ulcers treated with tadalafil were reviewed retrospectively.</div></div><div><h3>Results</h3><div>Eighty-two consecutive cases with anti-MDA5-antibody positive DM (anti-MDA5 DM) were identified, including 62 (75.6 %) female patients. Approximately 95.0 % of the patients had interstitial lung disease (ILD) and 45.1 % had rapidly progressive ILD (RP-ILD). All patients (100 %) had typical skin rashes, such as Gottron's sign (87.8 %), heliotrope sign (69.5 %), and the holster sign (24.4 %). Twenty-six patients (31.7 %) had cutaneous ulcers. Univariate analysis showed that ulcer patients were prone to Gottron's sign (<em>p</em> = 0.026), perlungual erythematosus (<em>p</em> = 0.007), and arthritis (<em>p</em> = 0.031), while shortness of breath after exercise (<em>p</em> = 0.027) and RP-ILD (<em>p</em> = 0.024) are more likely to appear in patients with non-skin ulcers. Among the anti-MDA5 DM patients with skin ulcers, 10 (38.5 %) were refractory cutaneous ulcers, including five male patients. After tadalafil was added, eight (80 %) patients showed improvement in the physician global assessment (PGA), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score and the patient's pain score using the visual analog score (p-VAS), within one to two months. In addition, the refractory cutaneous ulcers of five patients completely resolved after treatment.</div></div><div><h3>Conclusions</h3><div>This study found that ulcerative rash in anti-MDA5 DM may not be associated with RP-ILD. The results also suggest that tadalafil may be a useful therapy for refractory cutaneous ulcers.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152651"},"PeriodicalIF":4.6,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143139438","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}