{"title":"Discontinuation of belimumab in systemic lupus erythematosus patients who achieved disease stability: a retrospective cohort study.","authors":"Yanxia Ren, Tingting Yan, Chao Wang","doi":"10.1007/s10067-025-07571-0","DOIUrl":"https://doi.org/10.1007/s10067-025-07571-0","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the changes in various indicators of systemic lupus erythematosus (SLE) patients who discontinue belimumab after achieving disease stability, assess the potential impacts and risks of discontinuing belimumab, and provide scientific evidence and references for clinical decision-making.</p><p><strong>Methods: </strong>Pertinent data were retrospectively collected from SLE patients at the Second Affiliated Hospital of Jiaxing University who either discontinued or continued belimumab treatment after achieving disease stability. The study compared changes in disease activity, glucocorticoid usage, immunological markers, and other relevant indicators between the two groups after 6 months of follow-up.</p><p><strong>Results: </strong>After 6 months of follow-up, patients in the experimental group exhibited increased Systemic Lupus Erythematosus Disease Activity Index-2000 (SLEDAI-2000) scores, enhanced activity of immunological markers, a greater proportion of patients requiring increased glucocorticoid doses, and experienced new-onset systemic involvement. Notably, only 20% of these patients achieved low disease activity state (LLDAS). In contrast, patients in the control group experienced decreased SLEDAI-2000 scores, improved immunological markers, and a greater proportion requiring a reduction in glucocorticoid dosage, with 76.67% achieving LLDAS. The differences between the two groups were statistically significant (P < 0.05). No statistically significant difference was observed between the two groups concerning hospital readmissions due to disease activity, new organ damage, or the incidence of adverse events.</p><p><strong>Conclusions: </strong>Discontinuing belimumab in patients with stable SLE may lead to higher glucocorticoid requirements, enhanced activity of immunological markers, increased disease activity, and new-onset systemic involvement. Research in this area is necessary and can provide certain reference and guidance for clinical decision-making. Key Points • The subsequent conditions of SLE patients who discontinue belimumab after achieving disease stability warrant attention. • Discontinuing belimumab may lead to higher glucocorticoid requirements, enhanced activity of immunological markers, increased disease activity, and new-onset systemic involvement. • These findings provide crucial decision-making references and may help reduce unnecessary discontinuations.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682161","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":"Subtyping depression in the rheumatic diseases by cluster analysis.","authors":"Yaqi Zhao, Suyan Yan, Xinya Li, Wei Xu, Baocheng Liu, Zhenzhen Ma, Qingrui Yang","doi":"10.1007/s10067-025-07586-7","DOIUrl":"https://doi.org/10.1007/s10067-025-07586-7","url":null,"abstract":"<p><strong>Objective: </strong>Major depressive disorder (MDD) and rheumatic diseases (RD) interact to exacerbate disease outcomes. The purpose of this study was to assess the prevalence and associated factors of depression in RD patients in order to identify independent predictors of mental health disorders risk and apply cluster analysis to identify homogeneous groups in a population of approximately 47 patients with RD-MDD to achieve precise treatment and early prevention of complications.</p><p><strong>Methods: </strong>In total, 205 RD patients were included in this study. We used the Profile of Mood State (POMS) and Patient Health Questionnaire-9 (PHQ-9) to assess the patients' state of mind. A cluster analysis was applied according to six clinical and serological variables to define different subgroups of patients.</p><p><strong>Results: </strong>The rate of depression in RD patients in our study was 22.9%. Sex (female), disease duration, and disease activity are risk factors for the development of depression. Albumin is a protective factor for MDD. RD-MDD patients were clustered in two groups. Cluster 1 (n = 30, 63.8%): patients were of older age, lower education and income levels, low disease activity, and mild depressive symptoms. Cluster 2 (n = 17, 36.2%): Young women with higher education and income levels, high disease activity, and more severe depressive symptoms.</p><p><strong>Conclusion: </strong>Our findings provide evidence indicating that RD-MDD presents varying clinical phenotypes and the treatment varies accordingly, suggesting the need for individualized treatment. Key Points • Depression is often comorbid in patients with rheumatic diseases. The two interact and aggravate the patient's condition. • The rate of depression in RD patients in our study was 22.9%. Sex (female), disease duration, and disease activity are risk factors for the development of depression. Albumin is a protective factor for MDD. • RD-MDD patients were clustered in two groups through cluster analysis in order to guide individualized treatment.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682162","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}
Pietro Francesco Bica, Elvis Hysa, Rosanna Campitiello, Alberto Sulli, Carmen Pizzorni, Emanuele Gotelli, Sabrina Paolino, Ana Rebollo Gimenez, Clara Malattia, Marco Gattorno, Silvia Rosina, Vanessa Smith, Maurizio Cutolo
{"title":"Nailfold videocapillaroscopy in juvenile dermatomyositis: detailed correlations between microvascular abnormalities with clinical and laboratory parameters from an observational retrospective single-center study.","authors":"Pietro Francesco Bica, Elvis Hysa, Rosanna Campitiello, Alberto Sulli, Carmen Pizzorni, Emanuele Gotelli, Sabrina Paolino, Ana Rebollo Gimenez, Clara Malattia, Marco Gattorno, Silvia Rosina, Vanessa Smith, Maurizio Cutolo","doi":"10.1007/s10067-025-07561-2","DOIUrl":"https://doi.org/10.1007/s10067-025-07561-2","url":null,"abstract":"<p><strong>Introduction: </strong>Juvenile dermatomyositis (jDM) is a systemic autoimmune disease mainly affecting the skeletal muscle and skin. The aim of our study was to investigate the possible correlations between nailfold videocapillaroscopy (NVC) findings with clinical and laboratory parameters in patients with jDM.</p><p><strong>Methods: </strong>Twenty-eight jDM patients (mean age 7.1 ± 3 years, mean disease duration 3.1 ± 3 years), evaluated between 2019 and 2023, classified according to Bohan and Peter criteria, were age- and sex-matched with healthy controls. Clinical, laboratory, and NVC parameters were collected at first visit and after 1 year again in a subgroup of patients (n = 9).</p><p><strong>Results: </strong>Positivity for myositis-specific autoantibodies (MSA) was associated with a lower mean capillary density (p = 0.02) in jDM patients. Additionally, the presence of giant capillaries was significantly more frequent in MSA-positive patients (p = 0.04). The skin component of the disease activity score (VAS and DAS skin score) showed a significant inverse correlation with capillary density (r = -0.53, p = 0.007) and was significantly higher in patients with giant capillaries (median 5.5 vs 1, p = 0.005). Periungual telangiectasias and Gottron's papules were significantly associated with a reduction of the mean capillary count and with the presence of giant capillaries (all p-values < 0.05). Interestingly, no significant changes in NVC parameters were reported after 1 year of receiving immunosuppressive treatment.</p><p><strong>Conclusions: </strong>Capillary loss and the presence of giant capillaries at NVC analysis were significantly associated with clinical and laboratory markers of skin disease activity in jDM. These findings suggest that NVC may serve as a promising non-invasive tool to support disease assessment, although further prospective studies are needed to confirm its role in clinical practice. Key Points • Nailfold videocapillaroscopy (NVC) abnormalities, particularly capillary loss and giant capillaries, correlate significantly with skin disease activity in juvenile dermatomyositis (jDM). • Myositis-specific antibodies (MSA), especially anti-TIF1γ, are associated with more severe microvascular damage, suggesting their potential role in patient stratification. • NVC parameters remained stable over 1 year of immunosuppressive treatment, highlighting the need for longer-term studies to evaluate microvascular change progressions in jDM. • NVC may represent a promising non-invasive biomarker to support the clinical assessment and follow-up of patients with jDM, but further validation in larger prospective studies is warranted.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144674027","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}
Jian Huang, Lu Wang, Xiaodong Hu, Tianrui Wang, Yingze Zhang
{"title":"Genetic and molecular underpinnings of the link between rheumatoid arthritis and myasthenia gravis: Insights from GWAS and transcriptomic analyses.","authors":"Jian Huang, Lu Wang, Xiaodong Hu, Tianrui Wang, Yingze Zhang","doi":"10.1007/s10067-025-07582-x","DOIUrl":"https://doi.org/10.1007/s10067-025-07582-x","url":null,"abstract":"<p><strong>Background: </strong>Although studies have shown that patients with rheumatoid arthritis (RA) are at a higher risk of developing myasthenia gravis (MG), the causal relationship and shared genetic basis between these two diseases have not been fully investigated. The purpose of this study is to uncover the potential bidirectional causality between RA and MG, and to explore their shared genetic factors and possible pathogenic mechanisms.</p><p><strong>Methods: </strong>First, we utilized genome-wide association (GWAS) data from the IEU Open GWAS project, employing the online analysis platform MRBASE and applying four Mendelian randomization (MR) methods (Inverse Variance Weighted regression, Weighted Median, MR-Egger, and Weighted Mode) to explore the bidirectional causal relationship between RA and MG. Subsequently, we extracted transcriptomic data for RA and MG from the GEO database and used differential expression analysis, weighted gene coexpression network analysis (WGCNA), machine learning, and gene set enrichment analysis (GSEA) to identify key hub genes and their associated pathways. Furthermore, we employed the CIBERSORT method to analyze the immune cell infiltration in both diseases. Ultimately, based on these identified hub genes, we constructed a diagnostic model-nomogram-to aid in the diagnosis and prediction of the diseases.</p><p><strong>Result: </strong>RA is significantly associated with an increased risk of MG (Odds Ratio [OR]: 1.353, 95% Confidence Interval [CI]: 1.081 to 1.693, P = 0.008). However, there is insufficient evidence to support the hypothesis that MG increases the risk of RA. Through differential expression analysis and WGCNA methods, we collectively identified 18 key shared genes. Further, using two machine learning approaches, we ultimately identified 4 core hub genes (CDC42EP2, FKBP5, CD79A, and TDP1), which have great value in the diagnosis of RA and MG and are closely related to immune cell infiltration.</p><p><strong>Conclusion: </strong>Our study has unveiled the bidirectional causality between RA and MG, and identified shared molecular characteristics, highlighting the potential for developing targeted therapeutic strategies. Key Points • Our study shows a significant link between RA and increased MG risk, suggesting a bidirectional causal relationship. • We identified 18 key shared genes between RA and MG through differential expression and WGCNA, and pinpointed 4 core hub genes (CDC42EP2, FKBP5, CD79A, TDP1) using several machine learning algorithms. These genes are valuable for diagnosis and associated with immune cell infiltration. • We developed a diagnostic nomogram based on the hub genes, which could aid in diagnosing and predicting RA and MG, guiding clinical practice and personalized medicine.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144667251","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}
Rachael Stovall, Dominique Feterman Jimenez, Jose Andres Porres Arnaez, Vishwesh Bharadiya, Jean W Liew, Alison M Bays, Jenna Thomason, Londyn Robinson, Rashmi Dhital, Namrata Singh
{"title":"Patient characteristics and outcomes of emergency visits in psoriatic arthritis: results from the U.S. Nationwide Emergency Department Sample.","authors":"Rachael Stovall, Dominique Feterman Jimenez, Jose Andres Porres Arnaez, Vishwesh Bharadiya, Jean W Liew, Alison M Bays, Jenna Thomason, Londyn Robinson, Rashmi Dhital, Namrata Singh","doi":"10.1007/s10067-025-07577-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07577-8","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate patient characteristics and outcomes associated with emergency department (ED) visits in psoriatic arthritis (PsA) using the Nationwide Emergency Department Sample (NEDS), the largest all-payer U.S. ED database.</p><p><strong>Methods: </strong>We analyzed 2019 NEDS data for adults aged ≥ 18 years with and without PsA per International Classification of Diseases (ICD) codes. Baseline demographics, clinical comorbidities, and primary ED diagnoses were compared between PsA and non-PsA ED visits cross-sectionally. We assessed the top three primary diagnoses associated with inpatient admission among PsA ED visits. Pearson chi squared tests were used to compare categorical variables and t-tests to compare continuous variables. Multivariable logistic regression was used to identify factors associated with inpatient admission.</p><p><strong>Results: </strong>Among 117,359,429 ED visits, 61,079 (0.05%) involved PsA patients (mean age 58.9 ± 15.2 years, 59.6% female, 84.2% non-Hispanic White, 47.4% Medicare-insured). Compared to non-PsA visits, PsA visits involved older patients, higher Medicare usage (30.1% vs. 47.4%), and a greater admission frequency (54.6% vs. 17.0%). Top diagnoses linked to admission in PsA patients were septicemia, skin and subcutaneous infections, and heart failure. Factors increasing the odds of admission included older age (OR 1.02, 95% CI 1.02-1.03), alcohol use disorders (OR 3.43, CI 2.67-4.41), chronic kidney disease (OR 2.23, CI 1.89-2.64), obesity (OR 2.32, CI 2.05-2.64), and stroke (OR 4.83, CI 3.02-7.72).</p><p><strong>Conclusion: </strong>PsA ED visits were characterized by older, non-Hispanic White patients, with over half requiring admission. Differences in age and comorbidity burden between patients with and without PsA may explain some of the findings. Key Points • We used a large U.S. database to assess ED utilization and outcomes in PsA patients. • Older age, comorbidities, and socioeconomic factors influence inpatient admission odds in patients with PsA. • Findings highlight health disparities, emphasizing the need for interventions to improve PsA patient outcomes.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144667268","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}
Burak Oz, Yusuf Dogan, Ahmet Karatas, Suleyman Serdar Koca
{"title":"Radiological and serological predictors of cavitary transformation in rheumatoid arthritis-associated pulmonary nodules: a retrospective cohort study.","authors":"Burak Oz, Yusuf Dogan, Ahmet Karatas, Suleyman Serdar Koca","doi":"10.1007/s10067-025-07579-6","DOIUrl":"https://doi.org/10.1007/s10067-025-07579-6","url":null,"abstract":"<p><strong>Background/objectives: </strong>Cavitary pulmonary nodules (CPN) represent a rare but clinically relevant manifestation of rheumatoid arthritis (RA), yet their radiological and serological predictors remain poorly defined. This study aimed to identify key clinical, immunological, and imaging features associated with CPN among RA patients presenting with pulmonary nodules (PN).</p><p><strong>Methods: </strong>This retrospective cohort included 156 RA patients with PN identified on thoracic computed tomography between 2010 and 2024. Clinical data, autoantibody status, treatment characteristics, and radiological parameters were compared between patients with and without CPN.</p><p><strong>Results: </strong>CPN were detected in 9.0% (n = 14) of cases. Compared to patients without CPN, affected individuals had a significantly greater number of nodules, larger nodule diameters, and higher seropositivity for rheumatoid factor, anti-cyclic citrullinated peptide antibodies, and ANCA. The largest nodule diameter was the only independent predictor of cavitary transformation (OR: 1.59, 95% CI: 1.19-2.12; p = 0.002), with stronger effect sizes in penalized regression (OR: 6.69 per SD; 95% CI: 3.52-12.61). Despite limited sample size, ROC analysis identified an optimal cut-off of 18 mm, yielding excellent discriminative performance (AUC: 0.979; sensitivity: 92.9%, specificity: 95.8%), further supported by bootstrap validation. No significant differences in baseline treatments were observed, though greater variability in drug exposure and post-diagnostic therapy adjustments was noted in CPN-positive patients.</p><p><strong>Conclusions: </strong>Radiological morphology-particularly increased nodule size-alongside autoantibody positivity, are key correlates of cavitary transformation in RA-associated PN. These findings support the use of imaging-based risk stratification and highlight the need for prospective validation across broader RA populations. Key Points • CPN were detected in 9% of RA patients with PN on CT imaging • Larger PN diameter emerged as the principal independent predictor of cavitary transformation, with an18 mm cut-off providing high discriminative capacity (AUC = 0.979). • Seropositivity for RF, anti-CCP, and ANCA IFA was significantly associated with cavitation. • Greater heterogeneity in pre-diagnostic leflunomide exposure and more frequent post-diagnostictherapy modifications among CPN-positive patients suggest a dynamic and potentially treatmentinfluenceddisease pattern, possibly reflecting variability in therapeutic response over time.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144667269","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":"NETs promote fibroblast-like synoviocytes producing IL-6 to enhance T follicular helper cell response in rheumatoid arthritis.","authors":"Zijia Li, Yali Liu, Yan Zhang, Zhichun Zhang, Shangwen Lei, Haili Shen","doi":"10.1007/s10067-025-07543-4","DOIUrl":"https://doi.org/10.1007/s10067-025-07543-4","url":null,"abstract":"<p><strong>Objective: </strong>Although neutrophil extracellular traps (NETs) are recognized as key contributors to rheumatoid arthritis (RA) progression, their immunomodulatory effects on T follicular helper (Tfh) cell differentiation remain enigmatic. Based on the effect of NETs on RA fibroblast-like synoviocytes (RA-FLSs), our study is aimed at investigating the effect of NETs on Tfh cell differentiation in RA.</p><p><strong>Methods: </strong>Immunofluorescence, western blotting, and flow cytometry were used to investigate whether NETs mediated the inflammatory phenotype transformation of synovium fibroblasts to produce IL-6 and promote Tfh cell differentiation through TLR9/MyD88/NF-κB pathway. The expressions of Cit H3, MPO, TLR9, and Bcl-6 in the synovial tissues of the trauma control group and the RA group were analyzed by immunohistochemistry.</p><p><strong>Results: </strong>Our findings indicated that the plasma concentrations of IL-6 and MPO-DNA in RA patients were positively correlated with the level of IL-21 in lymphocytes and the proportion of circulating T follicular helper cells in peripheral blood. Further analysis revealed that NETs mediate the release of IL-6 from RA-FLSs through the TLR9/MyD88/NF-κB pathway, thereby inducing the differentiation of CD4 + T cells into Tfh cells and promoting the development of adaptive immunity in RA.</p><p><strong>Conclusion: </strong>This study demonstrated that NETs promoted Tfh cell differentiation by inducing IL-6 production by synovial fibroblasts through the TLR9/MyD88/NF-κB pathway. Targeting TLR9 may be a potential target for the treatment of RA. Key Points • NETs promoted Tfh cell differentiation by inducing IL-6 production by synovial fibroblasts through the TLR9/MyD88/NF-κB pathway. • Targeting TLR9 may be a potential target for the treatment of RA.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144667267","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 presentation of children with lupus nephritis from a low- and middle-income country (LMIC): an initial report from the Indian pSLE Nephritis Registry.","authors":"Sanjukta Poddar, Deblina Dasgupta, Subal Pradhan, Sangeetha Perungo, Mahesh Janarthanan, Kinnari Vala, Priya Pais, Susan Uthup, Jyoti Singhal, Suparna Guha, Sumantra Raut, Shakil Akhtar, Jigna Bathia, Suma Balan, Priyankar Pal, Rajiv Sinha","doi":"10.1007/s10067-025-07576-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07576-9","url":null,"abstract":"<p><strong>Introduction: </strong>Limited prospective data exist on pediatric LN (pLN) from low- and middle-income countries (LMIC), where ethnicity, socioeconomic factors, and healthcare access are likely to differ from high-income countries.</p><p><strong>Methods: </strong>The Indian Pediatric Lupus Nephritis registry has been running since 2020 across multiple centers in India. Children (≤ 18 years) diagnosed with lupus (as per 2012 SLICC criteria), presenting with nephritis, and confirmed by kidney biopsy are being prospectively enrolled. Clinical data, laboratory investigations, kidney biopsy results, and treatment responses have been documented prospectively. The current report documents their initial presentation.</p><p><strong>Results: </strong>A total of 154 children (75% female, median age 12 years-IQR 10-14 years) with biopsy-proven LN were enrolled by July 2024. Nearly two-thirds had LN at SLE diagnosis, and the rest developed within a maximum of 5 years of initial presentation. Common manifestations at presentation included edema (75%), hypertension (54%), and proteinuria (98%), of which 68% presented with nephrotic-range proteinuria. Acute kidney injury (AKI) was observed in 43%, with 20% in stage 3. Ninety-four percent of our cohort had low complements (C3, C4, or both), and 96% were ANA-positive. Class IV LN was the most common (45%) histopathological type and had significantly lower estimated glomerular filtration rate in comparison to Class V LN.</p><p><strong>Conclusion: </strong>Kidneys are often involved in the initial presentation of childhood lupus, and the majority have proliferative nephropathy leading to AKI, hypertension, and significant proteinuria. Children enrolled in the registry are under active follow-up to assess the renal responses which will help optimize the management of pLN in LMICs. Key Points •It is a well-known fact that kidney involvement is more common in pediatric lupus and is among one of the most important long-term prognostic factors. •There is scarcity of data on pediatric lupus nephritis (pLN) particularly from low- and middle-income countries (LMIC), and even among them, the majority of the studies are retrospective and limited by a small cohort size. •Through this prospective registry from a LMIC, we demonstrated that 2/3rd of children with lupus have kidney involvement at presentation and almost all (90%) develop LN within 2 years of the diagnosis of lupus. •Acute kidney injury (AKI) is known to increase mortality/morbidity risks independently. Many of the previous studies have under-reported AKI in pLN, probably because the data was collected retrospectively. On the other hand, we found AKI to be very common and to be present in about half of the cases at presentation.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642013","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}
Yi Wei, Yansong Huang, Xu Wang, Lina Zhang, Kunyu Zheng, Yunjiao Yang, Yanlei Yang, Chengmei He, Lin Qiao, Yongzhe Li, Fengchun Zhang, Li Wang
{"title":"Postpartum may be a risk factor for biochemical flares in patients with primary biliary cholangitis: A single-center experience.","authors":"Yi Wei, Yansong Huang, Xu Wang, Lina Zhang, Kunyu Zheng, Yunjiao Yang, Yanlei Yang, Chengmei He, Lin Qiao, Yongzhe Li, Fengchun Zhang, Li Wang","doi":"10.1007/s10067-025-07558-x","DOIUrl":"https://doi.org/10.1007/s10067-025-07558-x","url":null,"abstract":"<p><strong>Background and aims: </strong>Pregnancy and fetal outcomes in patients with primary biliary cholangitis (PBC) have garnered insufficient attention due to relatively rare in women of reproductive age. In this study, intricate relationship between PBC and pregnancy were investigated.</p><p><strong>Methods: </strong>Twenty pregnant patients with PBC under long-term follow-up in Peking Union Medical College Hospital were enrolled, which pregnancy and fetal outcomes were retrospectively analyzed.</p><p><strong>Results: </strong>Among the 28 pregnancies, 5 (17.9%) resulted in miscarriages, whereas 23 were live births (82.1%, including 21 full-term and two premature births). Notably, no post-mature births or stillbirths occurred. Adverse maternal events were observed in 6 cases (6/28, 21.4%), and adverse postpartum events occurred in 3 cases (3/28, 10.7%). Most patients with PBC (7/10, 70%) maintained relatively stable biochemical measures during pregnancy. However, up to 60% (6/10) of the patients experienced biochemical flares within the first 6 months postpartum. 4 patients continued to take ursodeoxycholic acid (UDCA) during pregnancy without encountering any adverse maternal or infant outcomes.</p><p><strong>Conclusions: </strong>Most patients with PBC (70%) were able to maintain stable biochemical parameters during pregnancy, with good maternal and infant outcomes. Nevertheless, 60% of the patients with PBC experienced biochemical flares within the first 6 months postpartum, so close monitoring is necessary. UDCA treatment appears to be safe during pregnancy. Key Points • This retrospective study focuses on peripartum safety in reproductive-age patients with PBC during pregnancy. • Although most patients with PBC could maintain biochemical stability during pregnancy, a significant number of patients experienced biochemical flares within the first 6 months postpartum. • UDCA treatment appears to be safe during pregnancy in patients with PBC.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648771","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}
Wenyan Xu, Xiudi Wu, Qiuxia Yu, Kefang Sun, Xinli Mao, Hua Ye, Lei Xu, Huogen Wang, Yidong Wan, Tejia Shen, Chunxiao Chen, Lan Li
{"title":"Predictors of anti-TNF treatment failure in intestinal Behçet's disease: a multicenter retrospective cohort study.","authors":"Wenyan Xu, Xiudi Wu, Qiuxia Yu, Kefang Sun, Xinli Mao, Hua Ye, Lei Xu, Huogen Wang, Yidong Wan, Tejia Shen, Chunxiao Chen, Lan Li","doi":"10.1007/s10067-025-07581-y","DOIUrl":"https://doi.org/10.1007/s10067-025-07581-y","url":null,"abstract":"<p><strong>Objective: </strong>Treatment options for intestinal Behçet's disease (BD) are limited. Despite the proven efficacy of infliximab and adalimumab, there is an urgent need to identify predictive factors for inadequate response or non-response to anti-TNF agents.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study involving 71 patients diagnosed with intestinal BD and treated with anti-TNF across four hospitals from August 2018 to December 2024. Demographic data, clinical symptoms, endoscopic findings, and laboratory parameters were collected at baseline and every 3 months after anti-TNF therapy until the latest follow-up appointment. The primary outcome was non-response or inadequate response to anti-TNF therapy, or presence of intestinal complications.</p><p><strong>Results: </strong>During the study period, 35.2% (25/71) of patients met the primary outcome at a median follow-up time of 8.5 months. Pre-treatment endoscopic score greater than 2.5, presence of opportunistic infections, DAIBD score > 12.5, ESR > 14.50 mm/h, and CRP > 12.83 mg/L three months post-treatment were identified to be associated with anti-TNF treatment failure. ROC curve established by incorporating these variables demonstrated a strong predictive capacity for treatment failure (AUC = 0.930). An internal validation of ROC curve was performed by the bootstrap method, which demonstrated good accuracy and stability. We subsequently developed a nomogram model to calculate the risk of treatment failure based on the above variables.</p><p><strong>Conclusions: </strong>Predictors of anti-TNF treatment failure in patients with intestinal BD included baseline endoscopic score, the occurrence of opportunistic infections, and DAIBD score, CRP and ESR at 3 months post-treatment. Our model can identify high-risk patients early, allowing for the timely optimization of treatment regimens. Key Points • We explored the predictive factors for inadequate response or non-response to anti-TNF agents in patients with intestinal Behçet's disease. • Treatment failure occurred in 35.2% of patients treated with anti-TNF at a median follow-up of 8.5 months. • Pre-treatment endoscopic score greater than 2.5, presence of opportunistic infections, DAIBD score over 12.5, ESR above 14.50 mm/h, and CRP above 12.83 mg/L at three months post-treatment are significant predictors of treatment failure.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642015","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}