Feng Chen, Xingyang Su, Fangyuan Yang, Jiangyan Yu, Chan Bai, Min Zhang
{"title":"The global burden and epidemiological trends of gout, particularly cases attributable to high Body Mass Index (BMI) in adolescents and young adults (aged 15-39 years): a secondary analysis from global burden of disease study 2021.","authors":"Feng Chen, Xingyang Su, Fangyuan Yang, Jiangyan Yu, Chan Bai, Min Zhang","doi":"10.1007/s10067-025-07494-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07494-w","url":null,"abstract":"<p><strong>Objectives: </strong>This study aims to analyze the burden and epidemiological trends of early-onset gout (EOG) and its association with high body mass index (BMI) in individuals aged 15 to 39 globally from 1990 to 2021.</p><p><strong>Methods: </strong>Using the Global Burden of Disease (GBD) 2021 data, global incidence, prevalence, disability-adjusted life years (DALYs), and DALYs attributable to high BMI were analyzed. We examined EOG burden across global, regional, and national levels, along with trends, decomposition, health inequality, and correlations with socio-demographic index (SDI).</p><p><strong>Results: </strong>In 2021, EOG accounted for 1,296,983 incident cases globally, with 5,077,197 prevalent cases and 170,599 DALYs, of which 54,909 were attributed to high BMI. The age-standardized incidence rate (ASIR) rose from 36.52 per 100,000 in 1990 to 43.60 per 100,000 in 2021. Significant regional variations were observed, with high-income North America having the highest incidence (115.02 per 100,000). EOG burden was higher in males. Additionally, the percentage of DALYs attributable to high BMI continues to rise. EOG burden growth was mainly due to population growth, while DALYs attributable to high BMI rose due to epidemiological shifts. Absolute inequality in EOG burden grew, but relative inequality remained moderate. DALYs attributable to high BMI were concentrated in regions with higher SDI.</p><p><strong>Conclusions: </strong>The global burden of EOG has increased from 1990 to 2021, with high BMI playing a significant role. Strengthened prevention and management of gout and high BMI in adolescents and young adults are necessary. Key Points • The study investigates the spatiotemporal trends of incidence, prevalence, and DALYs of early-onset gout (aged 15-39 years) globally over the past 30 years, as well as the impact of high BMI on its DALYs. • The findings reveal a significant increase in the global burden of early-onset gout from 1990 to 2021, with both the number and proportion of DALYs attributable to high BMI showing marked increases across different sexes, age groups, and SDI levels. • The burden of early-onset gout is distributed unevenly across regions and countries, with a clear correlation to SDI levels, and the burden is concentrated in countries with higher SDI.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Reyhan Bilici, Gizem Tuğçe Alp, Selma Özlem Çelikdelen, Mehmet Akif Öztürk, Murat Kekilli
{"title":"TNF ınhibitor resistance in ankylosing spondylitis: is Helicobacter pylori the overlooked culprit?","authors":"Reyhan Bilici, Gizem Tuğçe Alp, Selma Özlem Çelikdelen, Mehmet Akif Öztürk, Murat Kekilli","doi":"10.1007/s10067-025-07536-3","DOIUrl":"https://doi.org/10.1007/s10067-025-07536-3","url":null,"abstract":"<p><strong>Background: </strong>Ankylosing spondylitis (AS) is a chronic inflammatory disease that poses challenges in treatment due to resistance to anti-tumour necrosis factor (anti-TNF) therapies. This study investigates whether Helicobacter (H. pylori) infection contributes to reduced anti-TNF treatment response in AS patients.</p><p><strong>Methods: </strong>A cross-sectional study was conducted on 159 patients with AS. H. pylori infection was assessed using serological assays (ELISA for Iga and Igg), histopathological examination of gastric biopsies, and stool antigen testing. Disease activity and functional impairment were evaluated using ESR, CRP, BASFI, and ASDAS-CRP scores.</p><p><strong>Results: </strong>Patients with anti-TNF therapy resistance exhibited significantly higher H. pylori Iga seropositivity (p = 0.01) and histopathological positivity (p = 0.03). Additionally, they had longer anti-TNF treatment duration and higher inflammatory markers, including ESR, CRP, BASFI, and ASDAS-CRP scores, indicating a more significant inflammatory burden and functional impairment.</p><p><strong>Conclusion: </strong>Our findings suggest chronic H. pylori infection may contribute to anti-TNF therapy resistance in AS by promoting systemic inflammation and gut barrier dysfunction. These results underscore the importance of early, aggressive treatment strategies and suggest that H. pylori eradication may potentially enhance the efficacy of anti-TNF drugs. Future interventional studies are required to validate these findings and explore H. pylori screening as a therapeutic approach in AS management.</p><p><strong>Key points: </strong>• Resistance to anti-TNF therapy in AS presents a significant challenge, with the underlying mechanisms not yet fully understood. • This study identifies Helicobacter pylori (H. pylori) infection as a potential contributor to anti-TNF drug resistance in ankylosing spondylitis (AS), with Iga seropositivity and histopathological positivity being markedly higher in resistant patients. • Chronic H. pylori infection may promote systemic inflammation and gut barrier dysfunction, hindering immune responses to anti-TNF therapies. Patients exhibiting therapy resistance showed a more substantial inflammatory burden, including elevated ESR, CRP, BASFI, and ASDAS-CRP scores, which reinforces the connection between persistent inflammation and treatment failure. • Targeting H. pylori through screening and eradication could enhance the efficacy of anti-TNF drugs and improve treatment outcomes for patients with AS.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmed Elsaman, Shrouk Abdelmageed, Osama Sayed Daifallah
{"title":"Comparative efficacy of supra-scapular nerve block, posterior shoulder capsule hydro-dilatation, and shoulder interval hydro-dilatation in managing shoulder adhesive capsulitis.","authors":"Ahmed Elsaman, Shrouk Abdelmageed, Osama Sayed Daifallah","doi":"10.1007/s10067-025-07534-5","DOIUrl":"https://doi.org/10.1007/s10067-025-07534-5","url":null,"abstract":"<p><strong>Aim of work: </strong>This study aimed to compare the effects of supra-scapular nerve block, posterior intra-articular hydro-dilatation, and hydro-dilatation of the shoulder interval, in terms of improving pain, function, and range of motion in patients with adhesive capsulitis.</p><p><strong>Patients and methods: </strong>A total of 50 patients diagnosed with adhesive capsulitis were randomly divided into three groups. The first group received a suprascapular nerve block, the second group underwent posterior intra-articular hydro-dilatation, and the third group underwent shoulder interval hydro-dilatation. Patient assessment was conducted using visual analogue scale for pain, shoulder pain and disability index, and measurements of range of motion.</p><p><strong>Results: </strong>Group 1 experienced rapid and sustained pain reduction (p-value < 0.001 at both baseline vs. first follow-up and baseline vs. second follow-up), with non-significant improvement in internal rotation after 12 weeks (p value = 0.330). Group 2 showed delayed improvement in internal rotation (p-value = 0.068), but more sustained pain reduction (p-value < 0.001) and improved range of motion in all directions at the 12-week mark. Group 3 exhibited rapid pain reduction (p-value < 0.001) and improved range of motion, but non-significant improvements in internal (p-value = 0.131) and external rotation (p-value = 0.052) after 12 weeks.</p><p><strong>Conclusion: </strong>Although no significant differences were observed among the three groups, we recommend posterior intra-articular hydro-dilatation as it yielded the most promising and sustainable outcomes as regard pain reduction and range of motion improvement. Suprascapular nerve block is recommended for patients with prominent pain symptoms. Rotator interval hydro-dilatation is the least recommended intervention, being the most challenging and painful technique, and as it demonstrated a less sustained effect on range of motion. Key Points • This study aimed to find the best treatment modality for adhesive capsulitis through comparing the effects of suprascapular nerve block, posterior intra-articular hydro-dilatation, and hydro-dilatation of the shoulder interval. • Although no significant differences were found among three modalities, posterior intra-articular hydro-dilatation showed the best long-term outcomes for pain and range of motion. Suprascapular nerve block is recommended for patients with severe pain, while hydro-dilatation of the shoulder interval had less lasting benefits.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324672","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kyung-Ann Lee, Eunsun Oh, Seunghyun Kim, Hyun-Sook Kim
{"title":"Prevalence and progression of radiographic enthesopathy at hip and pelvis in patients with axial spondyloarthritis based on CT assessment.","authors":"Kyung-Ann Lee, Eunsun Oh, Seunghyun Kim, Hyun-Sook Kim","doi":"10.1007/s10067-025-07535-4","DOIUrl":"https://doi.org/10.1007/s10067-025-07535-4","url":null,"abstract":"<p><strong>Objective: </strong>This single‑center, retrospective study aimed to investigate the prevalence and progression of entheseal lesions at the hip and pelvis via computed tomography (CT) and determine the associated factors in patients with axial spondyloarthritis (axSpA).</p><p><strong>Methods: </strong>AxSpA patients who underwent CT including the pelvis and hip and age-sex-matched controls who underwent CT for genitourinary indications were analyzed. Eight bilateral entheseal sites were evaluated and scored as follows: 0 (no damage), 1 (irregularities and/or erosions), and 2 (enthesophytes). Agreement between readers for CT scoring was excellent (intraclass correlation coefficient, 0.673-1.000). To evaluate the longitudinal changes of radiographic enthesitis, additional CT for any reason after the baseline CT were also scored.</p><p><strong>Results: </strong>Ninety-one patients (mean age 36.6 ± 13.1 years) and 91 controls were analyzed. Enthesopathy was more frequently observed in the axSpA group (39.6%) compared to that in controls (21.9%) (P = 0.01), with the symphysis pubis (22.0%) most commonly affected. Entheseal scores of the symphysis pubis and greater trochanter were significantly higher in the axSpA group than those in controls. Smoking (OR, 3.653), modified Stoke Ankylosing Spondylitis Spinal Score (OR, 1.186), and use of TNF-alpha blockers (OR, 4.645) were independent factors associated with enthesopathy in axSpA patients. Radiographic progression of entheseal lesions over approximately 2 years was very rare.</p><p><strong>Conclusions: </strong>Radiographic enthesopathy of the pelvis and hip was prominent in axSpA patients and linked to spinal damage, smoking, and use of TNF-alpha blockers. Key points • Pelvic enthesopathy was more frequent in axSpA patients than in controls (39.6% vs. 21.9%). • The symphysis pubis was the most commonly affected entheseal site in axSpA patients. • Smoking, spinal damage, and TNF-alpha blockers were independent risk factors for enthesopathy • Radiographic progression of pelvic enthesopathy over two years was rare in axSpA patient.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Efficacy of rituximab versus cyclophosphamide in connective tissue disease‑related interstitial lung disease: a systematic review and meta-analysis.","authors":"Yan Liu, Yaoxiu Liu, Junlai Xu, Guoxing Zeng, Qingyuan Yang, Shuiming Xu","doi":"10.1007/s10067-025-07533-6","DOIUrl":"https://doi.org/10.1007/s10067-025-07533-6","url":null,"abstract":"<p><strong>Objective: </strong>This study systematically compares the efficacy and adverse events of rituximab (RTX) and cyclophosphamide (CYC) in patients with connective tissue disease-related interstitial lung disease (CTD-ILD).</p><p><strong>Methods: </strong>The EMBASE, Cochrane, and PubMed databases were systematically searched to find all relevant studies. Quality assessment, study selection, and data extraction were independently conducted by two reviewers. The mean changes in percentage of predicted forced vital capacity (FVC%) and percentage of predicted diffusing capacity for carbon monoxide (DLco%) of the patients were selected to be primary outcome measures. RevMan 5 software was used for the pooled analysis.</p><p><strong>Results: </strong>Among 1106 titles screened from multiple databases, six studies met the inclusion criteria (two randomized controlled trials and four retrospective observational studies). Patients of four studies were systemic sclerosis-related interstitial disease(SSc-ILD), one study was anti-synthetase syndrome-related interstitial lung disease (AsyS-ILD), and one study was CTD-ILD (included idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc) or mixed connective tissue disease (MCTD), rheumatoid arthritis(RA)). The summary weight mean difference of FVC% change in the RTX group compared with the CYC group was 0.86 (95% CI:-1.51,3.24; P = 0.48), and the summary weight mean difference of DLco% change in the RTX group compared with the CYC group was 6.43 (95% CI: 1.62, 11.23; P = 0.009). Our pooled analysis suggested no significant difference in FVC% improvement between RTX and CYC. RTX seems to be slightly superior to CYC in terms of DLco% improvement in our meta-analysis. However, only three out of six enrolled studies provided data on DLco% change. Therefore, the results for DLco% change should be cautiously interpreted. Studies enrolled showed that adverse events were fewer in the RTX group. RTX appears to offer a favorable balance between efficacy and safety.</p><p><strong>Conclusions: </strong>RTX demonstrated similar efficacy to CYC in improving lung function (FVC% and DLco%), with fewer adverse events.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144282733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Inflammatory cytokines mediate the gut microbiota-EGPA subtype link: a Mendelian randomization study.","authors":"Quanzhen Liu, Zijie Luo, Yanjuan Wu, Qiming Gan, Nuofu Zhang, Kang Wu","doi":"10.1007/s10067-025-07526-5","DOIUrl":"https://doi.org/10.1007/s10067-025-07526-5","url":null,"abstract":"<p><strong>Introduction: </strong>Limited studies have explored the potential link between gut microbiota and eosinophilic granulomatosis with polyangiitis (EGPA), and the specific inflammatory cytokines involved in EGPA remain controversial. Additionally, the causal relationships between gut microbiota, inflammatory cytokines, and EGPA subtypes, as well as whether inflammatory cytokines mediate these effects, are not well understood.</p><p><strong>Objectives: </strong>We aim to investigate the causal effects of gut microbiota and inflammatory cytokines on the subtypes of EGPA and to elucidate the mediating role of inflammatory cytokines in this relationship.</p><p><strong>Methods: </strong>We conducted a two-sample Mendelian randomization (MR) analysis using data from large-scale genome-wide association studies (GWAS) on gut microbiota, 91 inflammatory cytokines, and EGPA subtypes (ANCA/MPO ( +) EGPA and ANCA/MPO ( -) EGPA). A bidirectional MR analysis was performed to exclude the influence of EGPA subtypes on gut microbiota. Then, we explored the mediating role of inflammatory cytokines. The primary analysis employed the inverse-variance-weighted method, supplemented by MR-Egger, weighted median, weighted mode, and simple mode analyses. We used the Steiger test to address reverse causality, Cochran's Q statistic to evaluate heterogeneity, and the MR-PRESSO outlier detection test to identify and mitigate horizontal pleiotropy.</p><p><strong>Results: </strong>For ANCA/MPO ( +) EGPA, we identified 12 gut microbiota (5 positive, 7 negative) and three circulating inflammatory cytokines (1 positive, 2 negative) with causal effects. Specifically, the genus Ruminococcus1 was a potential risk factor (OR, 1.052; 95% CI, 1.019-1.087, P = 0.002), while Eubacterium nodatum showed a protective effect (OR, 0.979; 95% CI, 0.966-0.991, P = 0.0009). IL-1α was associated with an increased risk (OR, 1.029; 95% CI, 1.010-1.049, P = 0.003), whereas leukemia inhibitory factor had a mitigating effect (OR, 0.963; 95% CI, 0.942-0.985, P = 0.001). For ANCA/MPO ( -) EGPA, we identified seven gut microbiota (2 positive, 5 negative) and six circulating inflammatory cytokines (5 positive, 1 negative) with causal effects. The family Bacteroidaceae showed a protective effect (OR, 0.939; 95% CI, 0.894-0.986, P = 0.01), whereas Ruminococcus1 was a risk factor (OR, 1.057; 95% CI, 1.009-1.108, P = 0.02). IL-2 (OR, 1.043; 95% CI, 1.009-1.077, P = 0.011), FGF 19 (OR, 1.044; 95% CI, 1.016-1.073, P = 0.002), and CXCL11 (OR, 1.035; 95% CI, 1.010-1.060, P = 0.005) were linked to increased risk. We also observed that the genus Parasutterella may mediate risk for ANCA/MPO ( +) EGPA by reducing leukemia inhibitory factor, accounting for a 21.4% mediating effect. No significant heterogeneity of instrumental variables or horizontal pleiotropy was detected.</p><p><strong>Conclusion: </strong>This pioneering MR study bridges a significant gap by elucidating the relationship between gut microbi","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kateryna Yurchenko, Pil Højgaard, Redi Pecini, Sine S Korsholm, Axel C P Diederichsen, Jesper Lindhardsen, Eva Søndergaard, Amalie D Haue, Kasper Søltoft, Søren Jacobsen, Louise P Diederichsen
{"title":"Cardiac involvement in established idiopathic inflammatory myopathy assessed by cardiac magnetic resonance mapping.","authors":"Kateryna Yurchenko, Pil Højgaard, Redi Pecini, Sine S Korsholm, Axel C P Diederichsen, Jesper Lindhardsen, Eva Søndergaard, Amalie D Haue, Kasper Søltoft, Søren Jacobsen, Louise P Diederichsen","doi":"10.1007/s10067-025-07530-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07530-9","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the prevalence of subclinical, myocardial involvement in patients with established idiopathic inflammatory myopathies (IIM) compared to healthy controls using T1 and T2 mapping by cardiac magnetic resonance imaging (CMRI).</p><p><strong>Method: </strong>Fifty-five patients with established, stable IIM without overt cardiac involvement were consecutively enrolled in this cross-sectional study. All patients completed questionnaires, underwent clinical examination, blood tests including antibody profiling, electrocardiography, and CMRI with T1 and T2 mapping. Concurrently, CMRI was conducted on 19 healthy controls. Abnormal T1 and T2 values were defined as values exceeding the 95th percentile in the control group. Potential associations between abnormal T1 and T2 values and various cardiac- and IIM-related outcomes were assessed in exploratory analyses.</p><p><strong>Results: </strong>Abnormal T1 values were observed in 9% of all IIM patients, displaying significantly higher T1 values compared to healthy controls (p = 0.02). T2 values were elevated in 18% of IIM patients, particularly among patients with non-inclusion body myositis (IBM) IIM compared to healthy controls (p = 0.03). No significant associations between T1 or T2 values and cardiac or disease-related measures were found in the present cohort of IIM patients.</p><p><strong>Conclusions: </strong>Our study demonstrates that subclinical cardiac involvement may be present in established, stable IIM patients, with abnormal T1 and T2 mapping observed in up to 18% of the cohort. These findings underscore the importance of ongoing cardiac monitoring, even during stable phases of the disease. However, prospective studies are needed to determine the prognostic value of T1 and T2 mapping in this disease entity. Key Points • T1 and T2 mapping on cardiac MRI identify subclinical myocardial involvement in patients with IIM. • Subclinical myocardial involvement is present in up to 18% of patients with established, stable IIM. • Cardiac T1 and T2 abnormalities are rare in patients with IBM.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144282732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical and prognostic profiles in immune-mediated hypertrophic meningitis: a retrospective analysis of 92 cases.","authors":"Yifei Wang, Yuyan Yang, Xuemei Guo, Hui You, Manqing Xie, Shangzhu Zhang, Yunjiao Yang, Di Wu, Linyi Peng, Yunyun Fei, Xinping Tian, Mengtao Li, Yan Zhao, Xiaofeng Zeng, Wen Zhang, Jiaxin Zhou","doi":"10.1007/s10067-025-07531-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07531-8","url":null,"abstract":"<p><strong>Objectives: </strong>Immune-mediated hypertrophic pachymeningitis (HP) is an inflammatory disorder with diverse neurological manifestations. Considering its various etiology and rarity, HP remains a diagnostic challenge. Comparisons of clinical and prognostic profiles across different etiologies remain insufficiently explored. We analyzed the clinical characteristics, treatment approaches, and outcomes of immune-mediated HP patients from a single center in China.</p><p><strong>Method: </strong>A retrospective study was conducted at Peking Union Medical College Hospital, analyzing immune-mediated HP cases from December 2003 to September 2021. We evaluated patients' neurological symptoms, systemic involvement, image findings, and baseline laboratory results, and compared these features among three predominant etiologies: antineutrophil cytoplasmic antibody (ANCA)-related HP, idiopathic HP, and IgG4-related disease (IgG4-RD). The treatment approaches and outcomes were also reviewed.</p><p><strong>Results: </strong>The study included 92 patients, 49 (53.3%) male and 43 (46.7%) female. ANCA-related HP was the leading cause of immune-mediated HP (n = 42, 45.6%), followed by idiopathic HP (n = 28, 30.4%), IgG4-RD (n = 12, 13.0%), and other underlying causes (n = 10, 10.9%). Despite different etiologies, similar patterns of cranial nerve and brain region involvement were observed. Large doses of glucocorticoids combined with immunosuppressants were the most common treatment across all etiologies. Kaplan-Meier analysis (mean follow-up time: 4.1 years) showed no significant differences in relapse-free survival rates among the three subgroups.</p><p><strong>Conclusions: </strong>ANCA-related HP, idiopathic HP, and IgG4-RD HP were the three predominant causes of immune-mediated HP in this Chinese cohort. The three etiologies exhibited similar clinical manifestations, imaging findings, and long-term prognoses, indicating that a standardized management strategy may benefit HP patients regardless of etiology. Key Points • Immune-mediated hypertrophic pachymeningitis (HP) is a rare disorder with diverse etiologies, and research comparing clinical and prognostic profiles across different causes remains limited. • This study involved 92 immune-mediated HP patients, comparing clinical characteristics, imaging findings, treatment approaches, and outcomes among ANCA-related, idiopathic, and IgG4-related HP. • The three predominant etiologies of HP showed similar clinical features and similar relapse-free survival rates. • Regardless of the underlying etiology, HP patients may benefit from a uniform management approach.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical, radiologic, and serologic predictors of rheumatic disease in interstitial lung disease patients.","authors":"Tugce Bozkurt, Elif Dincses-Nas, Sevilay Batibay, Zeynep Nilufer Tekin, Esen Kasapoglu","doi":"10.1007/s10067-025-07505-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07505-w","url":null,"abstract":"<p><strong>Objective: </strong>Interstitial lung disease (ILD) can be the first manifestation of underlying rheumatic diseases. Identifying autoimmune features in ILD patients is crucial for early diagnosis and management. This study aims to evaluate the prevalence of rheumatic diseases in patients initially referred for ILD and to analyze their clinical, radiological, and serological characteristics.</p><p><strong>Methods: </strong>A total of 181 patients referred to the rheumatology outpatient clinic with suspected ILD, who had no known history of a rheumatologic disease, were retrospectively analyzed. Patients without chest CT/HRCT scans (n = 38) and those without a confirmed ILD diagnosis after radiological re-evaluation (n = 44) were excluded. Demographics, clinical symptoms, serology, and imaging findings were compared between groups.</p><p><strong>Results: </strong>Among the 99 ILD patients, 22 (22.2%) were diagnosed with a rheumatic disease following their ILD diagnosis. The most common rheumatic conditions were primary Sjögren's syndrome (n = 7), systemic sclerosis (n = 5), and rheumatoid arthritis (n = 5). The rheumatic disease-related ILD (RD-ILD) group had a significantly higher female predominance (77.3% vs. 34.7%, p < 0.001) and lower smoking prevalence (p = 0.006) compared to the non-RD-ILD group. Usual interstitial pneumonia was the most frequently observed chest CT/HRCT pattern in both groups. ANA, RF, and ACPA positivity was significantly higher in RD-ILD patients (p = 0.029, p = 0.003, and p = 0.001, respectively). Two patients met the IPAF classification criteria, both exhibiting NSIP patterns on chest CT/HRCT.</p><p><strong>Conclusion: </strong>A substantial proportion of ILD patients were subsequently diagnosed with a rheumatic disease, highlighting the importance of routine autoimmune screening in ILD patients. Female predominance, lower smoking rates, and higher serological positivity in RD-ILD patients suggest that early rheumatologic evaluation could facilitate timely diagnosis and management.</p><p><strong>Key points: </strong>• Interstitial lung disease may be the initial clinical sign of connective tissue diseases, highlighting the essential role of rheumatology in diagnosis and disease management.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Huan Zhang, Jiayi Deng, Yang Liu, Wen Xia, Lulu Zhuang, Houan Zhou, Zhengzhao Liu, Haitao Zhang, Weixin Hu
{"title":"Luminex-based pilot study identifies novel serum autoantibodies associated with disease activity in patients with lupus nephritis.","authors":"Huan Zhang, Jiayi Deng, Yang Liu, Wen Xia, Lulu Zhuang, Houan Zhou, Zhengzhao Liu, Haitao Zhang, Weixin Hu","doi":"10.1007/s10067-025-07528-3","DOIUrl":"https://doi.org/10.1007/s10067-025-07528-3","url":null,"abstract":"<p><strong>Objective: </strong>To explore the associations of novel serum autoantibodies with disease activity and organ injuries in patients with lupus nephritis (LN).</p><p><strong>Method: </strong>This study included 75 patients with active LN (5 class II, 37 class IV, and 33 class V). Indirect immunofluorescence was used to measure serum anti-dsDNA; ELISA was used to measure serum anti-PLA2R and anti-C1q, and the Luminex method was used to measure 58 novel serum autoantibodies, with a median fluorescence intensity (MFI)/threshold ≥ 1.5 as the antibody positivity cutoff.</p><p><strong>Results: </strong>The positivity rates for 11 novel antibodies (anti-PLA2R, anti-P2RY11, anti-DEXI, anti-HARS, anti-TUBA1B, anti-KRT8, anti-TUBB, anti-NCL, anti-HSPB1, anti-AGRN, and anti-LGALS8) were highly associated with SLE activity and were inconsistent with those for anti-dsDNA and anti-C1q. Anti-TUBA1B and anti-AGRN positivity may independently influence LN with extrarenal organ involvement (OR = 6.73, 95% CI = 1.19 ~ 38.09; OR = 6.11, 95% CI = 1.32 ~ 28.25, respectively). The positivity rate for anti-PLA2R as detected by the Luminex platform was 40%, whereas the rate among those detected by ELISA was 12.7%. Twenty-one patients who achieved renal remission were retested, and the positivity rates of anti-PLA2R (42.9% vs. 9.5%, P = 0.014), anti-KRT8 (47.6% vs. 4.8%, P = 0.001), and anti-AGRN (38.1% vs. 4.8%, P = 0.008) also significantly decreased from baseline.</p><p><strong>Conclusion: </strong>On the basis of Luminex technology, the present study revealed multiple novel autoantibodies related to SLE activity and organ injury, especially anti-PLA2R, anti-KRT8, and anti-AGRN, thereby providing novel serological markers for the assessment of activity in patients with LN. Anti-TUBA1B and anti-AGRN are strongly associated with extrarenal involvement. Key Points • The Luminex method is highly sensitive and high-throughput for detecting serum autoantibodies. • LN patients with anti-TUBA1B and anti-AGRN positivity are more likely to be complicated by extrarenal organ involvement. • Serum anti-PLA2R, anti-KRT8, and anti-AGRN reflect the activity of SLE and provide novel serological markers for the evaluation of LN patients negative for anti-dsDNA and anti-C1q.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}