{"title":"IgG4-Related Arterial Disease: An Unusual Case of Aortitis.","authors":"Martin Soubrier, Eric Hachulla","doi":"10.3899/jrheum.2024-0888","DOIUrl":"https://doi.org/10.3899/jrheum.2024-0888","url":null,"abstract":"","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":""},"PeriodicalIF":3.6,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142840048","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":"Reviewer Index 2024.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":"51 12","pages":"1269"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142774201","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}
Lisa Christopher-Stine, Anna Ciesluk, Hector Chinoy, Namita A Goyal, Kaniah Gunter, David Isenberg, Adrian Kielhorn, Ingrid E Lundberg, Tahseen Mozaffar, Sanjay Rakhade, Gerrit Vandenberg, Rohit Aggarwal
{"title":"The Dermatomyositis Disease Symptom Questionnaire (DM-DSQ): A Measure to Assess the Patient Experience of Dermatomyositis Symptoms.","authors":"Lisa Christopher-Stine, Anna Ciesluk, Hector Chinoy, Namita A Goyal, Kaniah Gunter, David Isenberg, Adrian Kielhorn, Ingrid E Lundberg, Tahseen Mozaffar, Sanjay Rakhade, Gerrit Vandenberg, Rohit Aggarwal","doi":"10.3899/jrheum.2023-1137","DOIUrl":"10.3899/jrheum.2023-1137","url":null,"abstract":"<p><strong>Objective: </strong>Dermatomyositis (DM) symptoms negatively affect the quality of life of individuals living with the disease. Disease-specific, patient-reported outcome (PRO) instruments are needed to assess symptoms important to individuals with DM. This study aimed to conceptualize patient DM experience and disease activity definition to refine the development of the Dermatomyositis Disease Symptom Questionnaire (DM-DSQ), a novel PRO instrument capturing patient-reported symptoms.</p><p><strong>Methods: </strong>An observational, qualitative study was conducted with 30 individuals with DM (aged ≥ 18 yrs) in the US. A 1-hour semistructured interview, including concept elicitation and cognitive debriefing, was conducted with each participant. Inductive coding was used to identify concepts; a saturation analysis was conducted to confirm sample size. Concepts from transcripts were used to refine the preliminary conceptual model and DM-DSQ items.</p><p><strong>Results: </strong>Concept elicitation analysis findings included disease symptoms (eg, muscle weakness) and functional impacts (eg, walking). The analysis achieved conceptual saturation; the first 5 interviews uncovered most of the concepts. During cognitive debriefing of the DM-DSQ, participants found the items relevant, comprehensive, and easily understood (except for \"skin sensitivity in sunlight\"). The revised DM-DSQ content appears preliminarily valid in the patient population surveyed, pending further additions and debriefing based on refinement of the preliminary conceptual disease model and items.</p><p><strong>Conclusion: </strong>The DM-DSQ is being used in a phase II clinical trial and could become a valuable tool for studies evaluating PROs in patients with DM. Preliminary results indicate its content validity; extensive psychometric analysis using clinical trial data will determine its ability to capture symptoms for patients with DM.</p>","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":"1198-1207"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141876551","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":"Tocilizumab (TCZ) for Giant Cell Arteritis: Clinical Outcomes Following Relapses and TCZ Discontinuation Due to Adverse Events.","authors":"Fumika N Nagase, Sho Fukui, Naoho Takizawa, Toshihiro Yamaguchi, Nobuhiro Oda, Hajime Inokuchi, Takanori Ito, Mitsuru Watanabe, Masei Suda, Yoichiro Haji, Yasuhiro Suyama, Ryo Rokutanda, Masahiro Minoda, Atsushi Nomura, Eishi Uechi, Hiromichi Tamaki","doi":"10.3899/jrheum.2024-0612","DOIUrl":"10.3899/jrheum.2024-0612","url":null,"abstract":"<p><strong>Objective: </strong>Tocilizumab (TCZ) is effective for giant cell arteritis (GCA). However, little is known regarding treatment modification and clinical outcomes after unfavorable events such as GCA relapses or TCZ discontinuation due to adverse events (AEs).</p><p><strong>Methods: </strong>This multicenter retrospective study included patients with GCA who initiated TCZ from 2008 to 2021 at 5 Japanese hospitals. GCA relapses and TCZ-related AEs were monitored for 2 years after TCZ initiation. In patients with GCA relapses, subsequent clinical courses, including relapse symptoms and treatment modification, were followed for 90 days after the relapses. Similarly, patients who discontinued TCZ because of AEs were additionally followed until 1 year after the TCZ discontinuation to evaluate AEs, relapses, and treatment changes.</p><p><strong>Results: </strong>Of 62 eligible patients, 10 patients (16%) relapsed after initiating TCZ therapy. Most relapses (8 of 10) occurred after extending TCZ intervals or discontinuing TCZ. Combinations of adjusting TCZ intervals, adjusting glucocorticoid (GC) dose, and/or adding or increasing methotrexate (MTX) therapy could manage the relapses without serious complications. In the entire cohort, AEs occurred in 28 patients (45%), and 8 patients (13%) discontinued TCZ because of AEs. After AE-related TCZ discontinuation, 6 patients attempted to taper GCs without other immunosuppressive therapy (IST), and 4 subsequently relapsed. In contrast, 2 patients who used other IST or biologic therapy could decrease GCs without relapses.</p><p><strong>Conclusion: </strong>Although GCA relapses can occur after initiating TCZ therapy, most relapses can be safely managed by adjusting TCZ, GC, and/or MTX doses. Adding IST or biologic treatments may potentially be related to preventing relapses when patients discontinue TCZ because of AEs.</p>","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":""},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142640142","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":"Association Between Proton Pump Inhibitors and the Risk of Intestinal Behçet Disease.","authors":"Keita Murakami, Junya Arai, Sozaburo Ihara, Yoshihiro Hirata, Yumi Tsuchida, Hirofumi Shoda, Mayo Tsuboi, Ken Kurokawa, Nobumi Suzuki, Hiroto Kinoshita, Yoku Hayakawa, Keishi Fujio, Mitsuhiro Fujishiro","doi":"10.3899/jrheum.2024-0442","DOIUrl":"10.3899/jrheum.2024-0442","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to investigate the association between proton pump inhibitor (PPI) use and the incidence of intestinal Behçet disease (BD) in patients with BD.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted at The University of Tokyo Hospital, including patients with BD diagnosed between April 2005 and November 2023. Cox models and Kaplan-Meier analyses were used to evaluate hazard ratios (HRs) and cumulative incidence of intestinal BD, respectively. Secondary analyses were performed to assess the duration and dose-response relationship of PPI use.</p><p><strong>Results: </strong>Among 194 patients with BD, 25.3% developed intestinal BD during a mean follow-up of 12 years. PPI users had a significantly higher incidence of intestinal BD compared to nonusers (adjusted HR 2.48, 95% CI 1.38-4.47, <i>P</i> = 0.002), with a confirmed duration/dose-dependent relationship. The cumulative incidence of intestinal BD was markedly elevated in PPI users (log-rank <i>P</i> < 0.001). The result was similar to that in the propensity score-matched cohort.</p><p><strong>Conclusion: </strong>This study demonstrates a significant association between PPI use and increased incidence of intestinal BD in patients with BD. Caution in prescribing PPIs for patients with BD is warranted due to the potential risk of severe complications associated with intestinal BD.</p>","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":"1193-1197"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141876539","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":"Prevalence, Predictors, and Prognosis of Serious Infections in Takayasu Arteritis: A Cohort Study.","authors":"Durga Prasanna Misra, Upendra Rathore, Swapnil Jagtap, Prabhaker Mishra, Darpan R Thakare, Kritika Singh, Tooba Qamar, Deeksha Singh, Juhi Dixit, Manas Ranjan Behera, Neeraj Jain, Manish Ora, Dharmendra Singh Bhadauria, Sanjay Gambhir, Vikas Agarwal, Sudeep Kumar","doi":"10.3899/jrheum.2023-1254","DOIUrl":"10.3899/jrheum.2023-1254","url":null,"abstract":"<p><strong>Objective: </strong>To describe the incidence, risk factors, and outcomes associated with serious infections in patients with Takayasu arteritis (TA).</p><p><strong>Methods: </strong>Serious infections, defined as infections resulting in hospitalization or death or unusual infections like tuberculosis, were identified from a cohort of patients with TA. Corticosteroid and disease-modifying antirheumatic drug (DMARD) use at the time of serious infection was noted. Demographic characteristics, clinical presentation, angiography, and disease activity at presentation, and the use of DMARDs during follow-up were compared between patients with TA with or without serious infections. Mortality in patients with TA who developed serious infections was compared to those who did not using hazard ratios (HR; with 95% CI).</p><p><strong>Results: </strong>Of 238 patients with TA, 38 (16%) had developed serious infections (50 episodes, multiple episodes in 8; 3 episodes resulted in death). Among the 38 initial episodes, 11/38 occurred in those not on corticosteroids and 14/38 in those not on DMARDs. Pneumonia (n = 19) was the most common infection, followed by tuberculosis (n = 12). Patients with TA who developed serious infections vs those who did not had higher disease activity at presentation (active disease 97.4% vs 69.5%, mean Indian Takayasu Arteritis Activity Score 2010 12.7 (SD 7.3) vs 10.2 (SD 7.0), mean Disease Extent Index in Takayasu Arteritis 11.2 (SD 6.1) vs 8.8 (SD 6.1) and were more frequently initiated on corticosteroids or DMARDs. HRs calculated using exponential parametric regression survival-time model revealed increased mortality rate in patients with TA who developed serious infections (HR 5.52, 95% CI 1.75-17.39).</p><p><strong>Conclusion: </strong>Serious infections, which occurred in the absence of immunosuppressive treatment in approximately one-fifth of patients with TA, were associated with increased mortality in patients with TA.</p>","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":"1187-1192"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140337398","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":"Children With Type I Interferonopathy: Commonalities and Diversities in a Large Patient Cohort.","authors":"Fatih Haslak, Huseyin Kilic, Sezgin Sahin, Busra Hotaman, Nur Memnune Cebi, Mehmet Yildiz, Amra Adrovic, Aybuke Gunalp, Elif Kilic Konte, Esma Aslan, Umit Gul, Nergis Akay, Yilmaz Zindar, Fitnat Ulug, Serhat Guler, Ayca Kiykim, Sezin Aydemir, Kenan Barut, Sema Saltik, Haluk C Cokugras, Ozgur Kasapcopur","doi":"10.3899/jrheum.2024-0294","DOIUrl":"10.3899/jrheum.2024-0294","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to provide a comprehensive overview of the clinical features, laboratory and screening results, treatment options, and outcomes of patients with type I interferonopathy. Our secondary goal was to identify the predictors of long-term morbidity or mortality.</p><p><strong>Methods: </strong>We included children with genetically confirmed type I interferonopathies, with a follow-up duration of > 1 year. Data were obtained retrospectively from medical records.</p><p><strong>Results: </strong>Of the 40 eligible patients for the study, 52.5% were female, with a median age of disease onset of 1.5 years (range 0.1-13.2 yrs). They were diagnosed at an average age of 6.8 (SD 4.6) years. Aicardi-Goutières syndrome was the most common diagnosis (n = 15, 37.5%). The central nervous system was the most frequently affected system (n = 27, 67.5%). Janus kinase inhibitors were administered to 17 (42.5%) patients. Twenty-five patients (62.5%) developed at least 1 permanent morbidity or died during follow-up; thus, they were included in the poor outcome group. Although younger age at disease onset, intracranial calcification (ICC), and lack of chilblains and elevated acute-phase reactants were significant in univariate logistic regression analysis, only ICC on magnetic resonance imaging at admission (adjusted odds ratio 19.69, 95% CI 1.08-359.05, <i>P</i> = 0.04) was found to be a significant predictor of poor outcomes in multivariate logistic regression analysis.</p><p><strong>Conclusion: </strong>For the first time, we evaluated the predictors of poor outcomes in patients with type I interferonopathy with a broad spectrum of subtypes. Further, our study's unique patient characteristics can provide valuable insights into these extremely rare conditions.</p>","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":"1208-1217"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141876542","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":"Evaluating and Refining Strategies for Rheumatoid Arthritis Prevention in First Nations Communities.","authors":"Sijia Liu, Ruwei Hu","doi":"10.3899/jrheum.2024-0726","DOIUrl":"10.3899/jrheum.2024-0726","url":null,"abstract":"","PeriodicalId":50064,"journal":{"name":"Journal of Rheumatology","volume":" ","pages":"1266-1267"},"PeriodicalIF":3.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142299770","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}