Fadi Kharouf, Pankti Mehta, Virginia Carrizo Abarza, Qixuan Li, Laura P Whittall Garcia, Dafna D Gladman, Zahi Touma
{"title":"Does baseline nephrotic range proteinuria determine the long-term outcomes of membranous lupus nephritis patients?","authors":"Fadi Kharouf, Pankti Mehta, Virginia Carrizo Abarza, Qixuan Li, Laura P Whittall Garcia, Dafna D Gladman, Zahi Touma","doi":"10.1016/j.semarthrit.2025.152756","DOIUrl":"10.1016/j.semarthrit.2025.152756","url":null,"abstract":"<div><h3>Objectives</h3><div>Management strategies for membranous lupus nephritis (MLN) are generally based on the severity of proteinuria. However, long-term outcomes comparing subnephrotic and nephrotic range proteinuria remain understudied. We explored whether baseline proteinuria level, subnephrotic or nephrotic, impacts long-term outcomes.</div></div><div><h3>Methods</h3><div>We conducted a retrospective study identifying patients with biopsy-proven MLN. Patients were categorized based on baseline proteinuria: subnephrotic (<3.5 g/day) or nephrotic (≥3.5 g/day). Long-term outcomes, including an adverse composite outcome (end-stage kidney disease, sustained ≥30 % decline in eGFR, or death) and LN flares, were analyzed. Time-to-event outcomes were assessed using Kaplan-Meier curves, and associations were evaluated using Cox regression.</div></div><div><h3>Results</h3><div>88 patients were included, with 49 (55.7 %) in the subnephrotic group (median 1.5 g/day) and 39 (44.3 %) in the nephrotic group (median 4.7 g/day). At baseline, the subnephrotic group had a longer time to LN onset, less frequent hyperlipidemia, higher serum albumin, less diffuse podocyte effacement, and less frequent cyclophosphamide treatment. No significant differences were noted in kidney function, urine sediment abnormalities, or histopathology. 38 patients (43.2 %) experienced the adverse composite outcome, with no difference between groups (40.8 % in the subnephrotic group vs. 46.2 % in the nephrotic group, <em>p</em> = 0.78]. Flares occurred in 35 patients (39.8 %), with no difference between groups (38.8 % in the subnephrotic group vs. 41.0 % in the nephrotic group, <em>p</em> = 1.00).</div></div><div><h3>Conclusions</h3><div>No significant differences in renal disease characteristics or long-term outcomes were found between MLN patients with nephrotic and subnephrotic baseline proteinuria. These findings challenge current practices, suggesting a need for more individualized immunosuppressive treatment in MLN.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152756"},"PeriodicalIF":4.6,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105725","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}
Elena Heras-Recuero , Antía García-Fernández , Teresa Blázquez-Sánchez , Cristina Gómez-Moreno , Iván Ferraz-Amaro , Javier Llorca , Miguel A González-Gay
{"title":"Strong correlation between SLEDAI and SLE-DAS in the Spanish population: Assessment of discordant patients","authors":"Elena Heras-Recuero , Antía García-Fernández , Teresa Blázquez-Sánchez , Cristina Gómez-Moreno , Iván Ferraz-Amaro , Javier Llorca , Miguel A González-Gay","doi":"10.1016/j.semarthrit.2025.152758","DOIUrl":"10.1016/j.semarthrit.2025.152758","url":null,"abstract":"<div><h3>Background</h3><div>Assessing disease activity in systemic lupus erythematosus (SLE) is essential for effective treatment. SLEDAI-2 K uses dichotomous items, while SLE-DAS incorporates both dichotomous and continuous variables,</div></div><div><h3>Objectives</h3><div>To analyze the correlation between SLEDAI-2 K and SLE-DAS in SLE patients from central Spain and analyze factors leading to discordance in disease activity classification.</div></div><div><h3>Methods</h3><div>Retrospective assessment of 324 SLE patients followed up from 2010 to 2024 at Madrid's Fundación Jiménez Díaz Hospital (Spain). Data were collected from the patients' most recent visits and disease activity was evaluated using SLEDAI-2 K and SLE-DAS, and discordant classifications between the tools were analyzed.</div></div><div><h3>Results</h3><div>The number of patients in each disease activity category was as follows: Remission (Clinical SLEDAI-2 <em>K</em> = 0, <em>n</em> = 254 [78.4 %] vs. clinical SLE-DAS =0, regardless of serology, and prednisone up to 5 mg/day, <em>n</em> = 253 [78.3 %]); Low activity (SLEDAI-2 K 1–4 and prednisone dose ≤ 5 mg/day, <em>n</em> = 42 [13.0 %] vs. SLE-DAS >0 and ≤ 2.48 with prednisone dose ≤ 7.5 mg/day, <em>n</em> = 14 [4.3 %]); Mild activity (SLEDAI-2 K 1–4 and prednisone dose > 5 mg/day or score 5–6, <em>n</em> = 19 [5.9 %] vs. SLE-DAS >0 and ≤ 2.48 with prednisone dose > 7.5 mg/day or score >2.48 and ≤7.64, <em>n</em> = 46 [14.2 %]); Moderate (SLEDAI-2 K 7–12 <em>n</em> = 7 [2.2 %] vs. SLE-DAS >7.64 and ≤9.9,<em>n</em> = 3 [0.9 %]); Severe SLEDAI-2 <em>K</em> > 12 (<em>n</em> = 2 [0.6 %] vs. SLE-DAS >9.9,<em>n</em> = 7 [2.2 %]). SLEDAI-2 K and SLE-DAS showed strong correlation (ρ=0.970, <em>p</em> < 0.001), with high concordance (linearly weighted Kappa index=0.7715, <em>p</em> < 0.001). Forty-four patients were discordant in terms of disease activity categorization. Of these, 39 were discordant at only one level of disease activity. Notably, in 37 of the 44 cases, SLE-DAS classified patients as having a higher degree of disease activity compared to SLEDAI-2 K. Patients with skin and hematological manifestations were more commonly discordant in terms of disease activity.</div></div><div><h3>Conclusion</h3><div>SLEDAI-2 K and SLE-DAS demonstrate a strong correlation and high reproducibility for assessing disease activity in the Spanish population. However, SLE-DAS offers additional information, particularly in patients with hematologic and skin involvement, enabling a more precise evaluation of disease activity in SLE patients</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152758"},"PeriodicalIF":4.6,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105724","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}
Yu Zhao , Gerald Chi , Shujun Xia , Xinchang Wang , Yongsheng Fan , Chenhang Ma , James Cheng-Chung Wei , Weijie Wang
{"title":"Infectious complications of Belimumab with standard care in systemic lupus erythematosus: a systematic review and meta-analysis","authors":"Yu Zhao , Gerald Chi , Shujun Xia , Xinchang Wang , Yongsheng Fan , Chenhang Ma , James Cheng-Chung Wei , Weijie Wang","doi":"10.1016/j.semarthrit.2025.152754","DOIUrl":"10.1016/j.semarthrit.2025.152754","url":null,"abstract":"<div><h3>Objectives</h3><div>We aimed to evaluate the risk of infectious complications of Belimumab with standard care in systemic lupus erythematosus (SLE).</div></div><div><h3>Methods</h3><div>We searched PubMed, Web of Science, EMBASE, and Cochrane databases for studies on SLE and Belimumab and evaluated the study quality using the Cochrane Collaboration tool (ROB 2). A random-effect model was employed to analyze the results. To evaluate publication bias, Egger's tests were used. We also performed subgroup analysis based on the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and British Isles Lupus Assessment Group(BILAG). The study protocol has been registered in PROSPERO (CRD42023421255).</div></div><div><h3>Results</h3><div>Eight studies involving 8545 patients were included, of which four studies had a high attrition bias. The present study suggested that the risks of infectious complications including infection (RR=1.02, 95 %CI=(0.97,1.08), I<sup>2</sup>=7.7 %) or serious infections (RR=0.94, 95 %CI=(0.77,1.15), I<sup>2</sup>=0 %), nasopharyngitis(RR=1.02, 95 %CI=(0.79,1.33), I<sup>2</sup>=36 %), upper respiratory tract infection(RR=0.97, 95 %CI=(0.83,1.14), I<sup>2</sup>=20.6 %), urinary tract infection(RR=1.09, 95 %CI=(0.91,1.32), I<sup>2</sup>=0 %), herpes zoster (RR=0.75, 95 %CI=(0.54,1.05), I<sup>2</sup>=0 %)and influenza(RR=0.98, 95 %CI=(0.69,1.39), I<sup>2</sup>=0 %) were not significantly increased in patients who received Belimumab with standard care, except for bronchitis (RR=1.51, 95 %CI=(0.98,2.33), I<sup>2</sup>=23.7 %). Additionally, the subgroup analysis of SLEDAI and the proportion of patients with BILAG 1A/2B did not show any significant impact on infectious complications.</div></div><div><h3>Conclusion</h3><div>Meta-analysis results demonstrated that intravenous (IV) Belimumab(≤10 mg/kg), along with standard care, did not significantly increase the risk of infectious complications in SLE patients having mild-to-moderate disease activity, except for bronchitis. Baseline disease activity and organ damage did not impact the risk of infectious complications.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152754"},"PeriodicalIF":4.6,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105722","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}
Sepehr Qooja , Matthew J Roberts , Nastaran Fakher , Mayada Demashkieh , Arumugam Moorthy , Nicolette C Bishop
{"title":"Is T-cell senescence associated with inflammatory arthritis and disease burden? A systematic review","authors":"Sepehr Qooja , Matthew J Roberts , Nastaran Fakher , Mayada Demashkieh , Arumugam Moorthy , Nicolette C Bishop","doi":"10.1016/j.semarthrit.2025.152757","DOIUrl":"10.1016/j.semarthrit.2025.152757","url":null,"abstract":"<div><div>Musculoskeletal and rheumatic diseases, such as rheumatoid arthritis (RA), axial spondyloarthritis (axSpA), and psoriatic arthritis (PsA), are prevalent and are increasingly common worldwide. The aetiology of these diseases varies; some are linked to mechanical stress (e.g., osteoarthritis), while others, like RA and axSpA, are associated with autoimmune processes. Despite their different origins, these conditions share a common feature in elevated inflammatory profiles compared to healthy populations. In autoimmune diseases, immune cells—particularly T-cells—are chronically activated, and the regulatory system is unable to control this. Chronic activation and proliferation often lead to a state known as cellular senescence. Senescent T-cells develop distinct characteristics, including resistance to apoptosis and the adoption of a pro-inflammatory senescence-associated secretory phenotype (SASP). This phenotype contributes to disease progression by driving the release of pro-inflammatory cytokines and elevating circulating levels of inflammatory markers. This systematic review summarises the results from 20 studies, out of 3203 that were initially picked up by the search phrase, to investigate whether levels of senescent T-cells are correlated with disease burden in the three mentioned conditions (RA, axSpA, and PsA). Our findings indicate that the level of senescent T-cells (T-helper CD4 cells and cytotoxic CD8 T-cells) is higher in patients when compared to healthy populations, and with their altered characteristics, these senescent cells could mechanistically contribute to disease progression, hence symptom burden. However, further investigation is needed in this field to show whether this increased level is associated with disease burden or not.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152757"},"PeriodicalIF":4.6,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105726","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}
Aleksandra R. Budarick , Cheryl L. Hubley-Kozey , Olga Theou , William D. Stanish , Meaghan Hannigan , Rebecca F. Moyer
{"title":"The effect of walking interventions on biomechanical knee osteoarthritis outcomes: A systematic review and meta-analysis","authors":"Aleksandra R. Budarick , Cheryl L. Hubley-Kozey , Olga Theou , William D. Stanish , Meaghan Hannigan , Rebecca F. Moyer","doi":"10.1016/j.semarthrit.2025.152755","DOIUrl":"10.1016/j.semarthrit.2025.152755","url":null,"abstract":"<div><h3>Purpose</h3><div>To summarize walking parameters specified in biomechanical analyses for knee osteoarthritis populations, determine the biomechanical effects of walking interventions, and explore associations between walking parameters and biomechanical knee osteoarthritis outcomes.</div></div><div><h3>Methods</h3><div>Databases (CINAHL, Embase, PubMED, SportDiscus, Scopus) were searched through October 2024. Experimental studies investigating biomechanical effects of walking interventions on knee osteoarthritis were included. Study quality was assessed using the <em>QualSyst</em> tool. Quantitative meta-analyses calculated Hedge’s g standardized mean differences (SMD) for first peak knee adduction moment (KAM), KAM impulse, peak knee flexion moment (KFM), and gait speed. Meta-regressions investigated the effect of walking parameters (intervention length; duration, frequency, intensity) on outcomes.</div></div><div><h3>Results</h3><div>Eighteen studies were included. Interventions investigated walking for 19.4 (SD=25.9) weeks, at 24.6 (SD=9.7) minutes per session, and 3.2 (SD=1.7) sessions per week. Most interventions specified self-selected intensity. Meta-analyses of 13 studies indicated walking interventions provide a very small increase in first peak KAM (SMD=0.18), no effect on KAM impulse (SMD=-0.01), small increase in peak KFM (adjusted SMD=0.23), and small increase in gait speed (SMD=0.35). Meta-regressions revealed longer interventions were associated with increased KFM (β=0.02), and higher walking frequency with increased gait speed (β=0.37). No other parameters were associated with biomechanical outcomes.</div></div><div><h3>Conclusions</h3><div>Walking interventions elicit minimal-to-no change in discrete biomechanical metrics of joint loading for individuals with mild-to-moderate knee osteoarthritis. Longer walking interventions or more frequent walking may provide additional functional benefit. These results may inform walking guidelines for knee osteoarthritis and predominantly support increased walking without detrimental effects to knee joint health.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152755"},"PeriodicalIF":4.6,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105721","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":"Peripartum maternal outcomes in individuals with systemic lupus erythematosus in a real-world electronic health record cohort","authors":"Saad Khan , Fatima Sohail , Hiba Thasleem , Junaid Imran , Maryam Adnan","doi":"10.1016/j.semarthrit.2025.152731","DOIUrl":"10.1016/j.semarthrit.2025.152731","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"74 ","pages":"Article 152731"},"PeriodicalIF":4.4,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144249469","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":"Toward a more actionable RA-ILD risk model: Insights beyond the KORAIL cohort","authors":"Yang Liu, Ying Liu, Qian Li, Ke Xu","doi":"10.1016/j.semarthrit.2025.152752","DOIUrl":"10.1016/j.semarthrit.2025.152752","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152752"},"PeriodicalIF":4.6,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144111831","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}
Sung Hae Chang , Eun Young Lee , Jeffrey A. Sparks
{"title":"Response to the correspondence by Xu et al. regarding \"Toward a more actionable RA-ILD risk model\"","authors":"Sung Hae Chang , Eun Young Lee , Jeffrey A. Sparks","doi":"10.1016/j.semarthrit.2025.152753","DOIUrl":"10.1016/j.semarthrit.2025.152753","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152753"},"PeriodicalIF":4.6,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094718","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 , Liam Harvey , Yasmin G. Hernandez-Barco , Zachary S. Wallace , Ana D. Fernandes , Grace A. McMahon , Isha Jha , Aubree E. McMahon , Cory A. Perugino , John H. Stone
{"title":"Defining pancreatic damage and symptom burden in IgG4-related autoimmune pancreatitis: A cross-sectional study of 118 patients from a single-center registry","authors":"Guy Katz , Liam Harvey , Yasmin G. Hernandez-Barco , Zachary S. Wallace , Ana D. Fernandes , Grace A. McMahon , Isha Jha , Aubree E. McMahon , Cory A. Perugino , John H. Stone","doi":"10.1016/j.semarthrit.2025.152742","DOIUrl":"10.1016/j.semarthrit.2025.152742","url":null,"abstract":"<div><h3>Objectives</h3><div>Type 1 autoimmune pancreatitis is a common manifestation of IgG4-related disease (IgG4-RD). However, there is a paucity of literature characterizing pancreatic damage and symptom burden in IgG4-RD.</div></div><div><h3>Methods</h3><div>We performed a cross-sectional analysis of patients who fulfilled the ACR/EULAR IgG4-RD Classification Criteria. Disease features and complications were collected by medical record review. A survey regarding symptoms and disease history was distributed to all patients. Characteristics were compared between patients with and without autoimmune pancreatitis.</div></div><div><h3>Results</h3><div>Of 303 patients who fulfilled Classification Criteria at the time of the chart review, 118 (39 %) had evidence of autoimmune pancreatitis. Overt indicators of acute pancreatitis (e.g., abdominal pain, nausea/emesis, elevated serum lipase) each occurred in fewer than 50 % of patients with autoimmune pancreatitis. Diabetes mellitus (DM), exocrine pancreatic insufficiency (EPI), or both were present in 47 %, 48 %, and 21 % of the autoimmune pancreatitis patients, respectively. After encouraging all patients to have fecal elastase measured, 40/49 (82 %) stool samples had low elastase concentrations. 9/118 (8 %) had undergone pancreatic resections before the diagnosis was established. 162/325 (50 %) completed surveys (<em>n</em> = 81 [50 %] with autoimmune pancreatitis). Patients with autoimmune pancreatitis reported a higher burden of abdominal pain, weight loss, and changes in stool than those without (all <em>p</em> < 0.05).</div></div><div><h3>Conclusion</h3><div>Despite an often subclinical presentation, autoimmune pancreatitis is associated with EPI, DM, or both in a high percentage of patients with IgG4-RD. While symptomatic acute pancreatitis may not be common, patient-reported symptom burden due to IgG4-related autoimmune pancreatitis or its complications is greater than previously appreciated.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152742"},"PeriodicalIF":4.6,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105720","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":"Variability in phenotype clusters of Behçet’s syndrome: A systematic review","authors":"Betul Macit , Sinem Nihal Esatoglu , Kevser Akyuz-Yesilyurt , Gulen Hatemi","doi":"10.1016/j.semarthrit.2025.152744","DOIUrl":"10.1016/j.semarthrit.2025.152744","url":null,"abstract":"<div><h3>Background</h3><div>Behçet’s syndrome (BS) is a multisystem vasculitis, and distinct clinical phenotypes with clustering of certain organ manifestations were proposed. However, studies from different cohorts have shown variability in the defined phenotypes. This was attributed to geographic and ethnic differences, but different studies from the same country have also shown variability in phenotype clusters. We aimed to explore the variability in clinical phenotype clustering across different countries and cohorts and possible reasons for these.</div></div><div><h3>Methods</h3><div>An electronic search was carried out in PubMed, EMBASE, and Cochrane Library for studies that assessed phenotype clusters in BS cohorts. Two reviewers independently performed the screening of titles, abstracts, and full texts using Covidence.</div></div><div><h3>Results</h3><div>A total of 15 studies that assessed 17 different cohorts were identified. Several differences were identified in the clusters that were reported in these cohorts. Factors that were identified by this systematic review as possible causes of these differences were study design, statistical analysis method (hierarchical cluster analysis vs. factor analysis), patient population (pediatric vs. adult), setting, diagnostic/classification criteria (International Study Group vs. International Criteria for Behçet’s Disease), disease duration, the definition of organ involvement (such as including cerebral sinus thrombosis in nervous system or vascular involvement), ascertainment of manifestations (such as gastrointestinal involvement confirmed by endoscopy or not), and time component for clustering of manifestations.</div></div><div><h3>Conclusion</h3><div>There is important variability in the phenotype clusters that are reported in different studies and this variability seems to stem from methodologic differences between the studies.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"73 ","pages":"Article 152744"},"PeriodicalIF":4.6,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143947527","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}