Clinical Rheumatology最新文献

{"title":"Coronary periarteritis in IgG4-RD: A case series.","authors":"Georges El Hasbani, Daniel Larson, Matthew J Koster, Kenneth J Warrington","doi":"10.1007/s10067-025-07684-6","DOIUrl":"https://doi.org/10.1007/s10067-025-07684-6","url":null,"abstract":"<p><strong>Objectives: </strong>IgG4-related disease (IgG4-RD) can affect multiple organ systems, with coronary artery involvement being rare. Coronary periarteritis may lead to complications such as myocardial infarction and ischemic cardiomyopathy. This case series characterizes the clinical and radiological features, complications, and treatment strategies in patients with IgG4-RD-associated coronary periarteritis.</p><p><strong>Methods: </strong>A retrospective review of the Mayo Clinic electronic health record identified 146 patients, of whom 9 met the inclusion criteria for IgG4-RD with coronary periarteritis. Clinical, laboratory, imaging, treatment, and outcome data were analyzed.</p><p><strong>Results: </strong>The cohort included 9 male patients (mean age: 55.5 ± 5.7 years). The median serum IgG-4 level was 282 mg/dL (interquartile range [IQR], 148-393), while the median C-reactive protein value was 10.8 mg/dL (IQR 7.5-53.6). The mean time from IgG4-RD diagnosis to coronary involvement was 2.5 years. CT angiography detected vessel wall thickening, affecting the right coronary artery (RCA) in five cases, the left anterior descending artery (LAD) in four, and the left main coronary artery in one. Aneurysm formation occurred in two cases. All patients received glucocorticoids, and seven were treated with rituximab. Over a mean follow-up of 7.5 years, one patient developed ischemic heart disease, and another progressed to heart failure. Repeat imaging showed partial or complete resolution of coronary thickening in seven patients.</p><p><strong>Conclusion: </strong>Coronary artery involvement in IgG4-RD is rare but may lead to aneurysm formation and ischemic complications. CT and MRI are valuable diagnostic tools, and rituximab with glucocorticoids appears effective, though further studies are needed. Key Points • IgG4-related disease can involve the coronary arteries, leading to periarteritis and potential complications such as myocardial infarction and aneurysm formation, highlighting the need for early recognition. • Glucocorticoids and rituximab appear to be effective in managing IgG4-RD-associated coronary periarteritis, but further studies are needed to evaluate long-term outcomes.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145033000","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":"Knowledge mapping of plasmacytoid dendritic cells in systemic lupus erythematosus: a bibliometric analysis (2004-2023).","authors":"Yuan Tian, Kang Tao, Hongyan Zhou, Shifei Li, Xiaoqiang Chen, Mingwang Zhang, Zhifang Zhai","doi":"10.1007/s10067-025-07674-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07674-8","url":null,"abstract":"<p><strong>Background: </strong>Plasmacytoid dendritic cells (pDCs) are a specialized subset of dendritic cells known for their ability to produce type I interferon (IFN I), contributing to antiviral defense and the pathogenesis of autoimmune diseases like systemic lupus erythematosus (SLE). In SLE patients, pDCs are excessively activated, leading to overproduction of IFN-α, which plays a critical role in disease progression. However, no bibliometric analysis has been conducted on the relationship between pDCs and SLE. This study aims to map the knowledge structure and trends in research on pDCs in SLE through bibliometric analysis.</p><p><strong>Methods: </strong>We analyzed publications related to pDCs in SLE from 2004 to 2023 using the Web of Science Core Collection. Data were analyzed and visualized with VOSviewers, CiteSpace, and the R package bibliometrix, offering a comprehensive view of the research landscape.</p><p><strong>Results: </strong>The analysis included 1086 articles from 57 countries, with the USA leading in publication volume. Major contributing institutions include Uppsala University, University of Michigan, and Baylor Institute for Immunology Research. Among the 565 authors, Ronnblom Lars was the most prolific, while Elkon Keith B ranked highest in terms of network centrality. Key research themes focus on pDC mechanisms in SLE development and emerging therapeutic strategies. Trending topics include \"anifrolumab,\" \"mortality,\" and \"population.\"</p><p><strong>Conclusion: </strong>This bibliometric study provides a thorough overview of research trends on pDCs in SLE, identifying recent advances and key areas of interest, offering valuable insight for future research directions.</p><p><strong>Key points: </strong>• pDCs play a crucial role in systemic lupus erythematosus (SLE) through the overproduction of type I interferon (IFN-α). • This is the first bibliometric analysis mapping the research landscape of pDCs in SLE from 2004 to 2023. • Emerging research focuses on therapeutic strategies, including trending topics like \"anifrolumab\" and \"mortality\" in SLE. • This study offers valuable insights into key research themes and trends, guiding future investigations on pDCs in SLE.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145014033","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 characteristics of dyslipidemia in patients with primary biliary cholangitis: a single-center experience in China.","authors":"Xu Wang, Tihong Shao, Ran Tian, Jia Liu, Yi Wei, Li Wang, Fengchun Zhang","doi":"10.1007/s10067-025-07662-y","DOIUrl":"https://doi.org/10.1007/s10067-025-07662-y","url":null,"abstract":"<p><strong>Aims: </strong>Dyslipidemia is frequently observed among individuals diagnosed with primary biliary cholangitis (PBC), though its specific characteristics remain incompletely defined. This study aimed to examine the lipid profile patterns and medical features of dyslipidemia in people suffering from PBC.</p><p><strong>Methods: </strong>Following the classification criteria proposed by the National Lipid Association, dyslipidemia is classified on the basis of abnormal plasma concentrations of high-density lipoprotein cholesterol (HDL-C), total cholesterol (TC), triglycerides (TG), and low-density lipoprotein cholesterol (LDL-C). Clinically, it is categorized into simple high TC, simple high TG, mixed hyperlipidemia, simple HDL-C decrease, and simple high LDL-C. In total, 257 individuals diagnosed with PBC who had dyslipidemia were enrolled in the study, along with 78 age- and sex- matched patients with simple dyslipidemia as the comparison group.</p><p><strong>Results: </strong>In contrast to the control group, patients with PBC exhibited markedly increased concentrations of HDL-C, TC, and TG, while LDL-C levels were somewhat reduced. Analysis revealed that HDL-C, TC, and LDL-C levels were positively correlated with liver enzyme levels, whereas TG levels showed a negative correlation with bilirubin levels. In terms of clinical classification, patients with PBC had higher rates of simple TC increase and mixed hyperlipidemia, but a lower proportion had isolated LDL-C increase compared with controls. Patients with PBC who had simple high TC and mixed hyperlipidemia showed higher liver enzyme levels than those with simple high TG, while clinical symptoms were similar across groups.</p><p><strong>Conclusion: </strong>Dyslipidemia in PBC exhibits unique characteristics, indicating a distinct pathogenesis compared to conventional dyslipidemia. These findings highlight the potential benefit of proactive lipid management in supporting liver function. Key Points • Compared with patients who have simple dyslipidemia, dyslipidemia in patients with PBC has distinct characteristics. • Dyslipidemia is clinically associated with liver damage, and correlation analysis showed a relationship between blood lipid levels and liver function.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144999867","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":"Reduced TIGIT⁺CD56⁺NK cells associate with disease progression and impaired immune regulation in primary Sjögren's syndrome.","authors":"Ping Zhao, Saizhe Song, Wei Cheng, Cheng Peng, Junrong Wang, Xin Chang, Jian Wu, Zhongli Hu, Cuiping Liu","doi":"10.1007/s10067-025-07665-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07665-9","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to explore the expression and clinical significance of TIGIT⁺CD56⁺NK cells within the peripheral blood of patients with primary Sjögren's syndrome (pSS) in this study, as well as to examine the role of TIGIT in modulating NK cell function in individuals with pSS.</p><p><strong>Methods: </strong>The percentage of TIGIT⁺CD56⁺NK cells was detected in the peripheral blood of 76 individuals with pSS and 63 healthy controls (HCs) using flow cytometry. The percentage of TIGIT⁺CD56⁺NK cells across various clinical features, laboratory parameters, and between active and inactive patients was analyzed. Subsequently, we analyzed the relativity between the percentage of TIGIT⁺CD56⁺NK cells and the clinical parameters in pSS patients. The percentage of TIGIT⁺CD56⁺NK cells in 10 patients with pSS was observed before and after treatment. Furthermore, we constructed receiver operating characteristic (ROC) curves to assess the diagnostic value of the percentage of TIGIT⁺CD56⁺NK cells in pSS and to predict the disease activity of pSS. We further detected the levels of cytokines secreted by TIGIT⁺ and TIGIT^-NK cells in 5 pSS patients to assess the function of NK cells.</p><p><strong>Results: </strong>In patients with pSS, there was a reduction in the percentage of TIGIT⁺CD56⁺NK cells, particularly among those with active disease. The percentage of TIGIT⁺CD56⁺NK cells exhibited a significant reduction in patients with pSS accompanying xerostomia, decayed tooth, glandular swelling, fatigue, arthralgia, cutaneous manifestations, renal tubular acidosis (RTA), interstitial lung disease (ILD), leukopenia, lymphopenia, increased serum globulin, increased ESR, increased IgG, increased IgA, and positive tests for anti-Ro52, anti-Ro60, and rheumatoid factor (RF), compared to those with negative results. TIGIT expression on CD56⁺ NK cells exhibited a negative relevance with ESR, serum globulin levels, RF, IgG levels, ESSDAI scores, and ESSPRI scores. After treatment, the TIGIT⁺CD56⁺NK cells percentage increased notably. The ROC curve demonstrated that the level of TIGIT⁺CD56⁺NK cell percentage exhibited an excellent capacity for differentiating pSS and predicting disease activity. The expression levels of CD69, Ki67, perforin, tumor necrosis factor-α (TNF-α), and interferon-γ (IFN-γ) were found to be lower in TIGIT⁺CD56⁺NK cells compared to TIGIT^-CD56⁺NK cells among patients with pSS. Furthermore, we discovered that expression levels of perforin and TNF-α were negatively related with that of TIGIT⁺CD56⁺NK cells.</p><p><strong>Conclusion: </strong>Our research indicated that a reduction in TIGIT⁺CD56⁺N","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144991632","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":"The relationship between vitamin D receptor (VDR) genomic polymorphisms, disease activity, and functional ability in patients with psoriatic arthritis.","authors":"Alen Vrtaric, Simeon Grazio, Marija Bozovic, Tomislav Pavicic, Hana Skala Kavanagh, Ines Doko Vajdic, Frane Grubisic, Lucija Prtenjaca, Nora Nikolac Gabaj","doi":"10.1007/s10067-025-07610-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07610-w","url":null,"abstract":"<p><strong>Introduction/objectives: </strong>Psoriatic arthritis (PsA) is a chronic inflammatory rheumatic disease strongly associated with psoriasis. We hypothesized that the presence of variant alleles in VDR may be associated with worse clinical outcomes. The aim of this study was to evaluate a possible association between the FokI and TaqI polymorphisms in the VDR gene and clinical markers of disease activity and functional status in PsA.</p><p><strong>Method: </strong>Study included 68 patients with PsA. Diagnosis was confirmed according to fulfillment of CASPAR criteria for PsA. Disease activity for peripheral arthritis was evaluated using the DAPSA index; global assessment of disease activity and pain were assessed using VAS. BASDAI was used for the axial form of PsA. Functional ability was assessed by HAQ-DI and FACIT-F. BASFI was used for measuring the functional status of axial involvement. Overall functional mobility was assessed with TUG. Laboratory tests included genotyping of the FokI (rs2228570) and TaqI (rs731236), as well as CRP, ESR, IL-6, and 25(OH)D3. Significance was set at P < 0.05.</p><p><strong>Results: </strong>Significant difference in FokI polymorphism was observed for intensity of pain, with the T/T genotype reporting worse outcomes than C/C (P = 0.049). BASDAI showed significant differences (P = 0.026), with T/T genotype having higher values than C/C genotype.</p><p><strong>Conclusions: </strong>FokI polymorphism may have a potential role in modulating disease activity and pain perception in patients with PsA, whereas the TaqI polymorphism appears to have less impact on clinical outcomes. The findings emphasize the need for further research to better understand the genetic impact on disease severity in PsA.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144999841","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":"Time to incident ocular and extraintestinal manifestation in HLA-B27-Associated diseases: a comparative study of immunotherapies.","authors":"Negin Yavari, Karen M Wai, Amer F Alsoudi, Euna Koo, Chase A Ludwig, Andrea L Kossler, Quan Dong Nguyen, Prithvi Mruthyunjaya, Ehsan Rahimy","doi":"10.1007/s10067-025-07670-y","DOIUrl":"https://doi.org/10.1007/s10067-025-07670-y","url":null,"abstract":"<p><strong>Objectives: </strong>This study compared the incidence and time-to-event outcomes of ocular extraintestinal manifestations (O-EIMs) and EIMs among patients with human leukocyte antigen (HLA)-B27-associated diseases receiving different classes of immunotherapy.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted using aggregated electronic health records from the TriNetX network between January 1, 2014, and December 31, 2024. Patients with HLA-B27-associated diseases were included if they were newly prescribed tumor necrosis factor (TNF), janus kinase (JAK), or interleukin (IL) inhibitors on or after their initial diagnosis. The cumulative incidence of O-EIMs and EIMs was calculated per 100 person-years. Kaplan-Meier estimates were used to graph time-to-event outcomes using R Studio (version 4.4.1).</p><p><strong>Results: </strong>We identified 22,287 patients [mean age 47.2 (16.8) years; 13,132 (58.9) % women]. The cumulative incidence of anterior uveitis from the index date was 0.11 cases per 100 person-years. According to first medication prescribed, the incidence of anterior uveitis was lowest in patients prescribed IL inhibitors, followed by JAK and TNF inhibitors (0.07%, 0.10%, and 0.20%, cases per 100 person-years respectively). Time-to-event analysis showed a higher likelihood of anterior uveitis with initial TNF inhibitor use, although the difference was not statistically significant. Regarding EIMs, incident sacroiliitis was highest with TNF (0.91%), followed by JAK (0.72%) and IL (0.37%). Psoriatic dermatitis incidence was highest with JAK inhibitors (2.85%), compared to TNF (2.19%) and IL (2.17%).</p><p><strong>Conclusion: </strong>In this cohort study of patients with HLA-B27-associated diseases, the lack of statistical significance indicates similar effectiveness across immunotherapy classes in preventing O-EIMs and EIMs. Key Points • Large-scale comparative study assessing time-to-incident O-EIMs and EIMs in patients with HLA-B27-associated diseases treated with TNF, JAK, or IL inhibitor therapies using real-world data from the TriNetX network. • Low overall incidence of O-EIMs and EIMs was observed across all immunotherapy classes, with no statistically significant difference in time-to-event outcomes, indicating similar effectiveness in preventing these manifestations.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144991615","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":"Exploring rituximab efficacy in systemic lupus erythematosus with pulmonary hemorrhage: a retrospective case series from a single center.","authors":"Jianfen Meng, Jinchao Jia, Ke Gan, Qichen Gao, Longfang Chen, Dehao Zhu, Mengyan Wang, Yu Xiao, Yuning Ma, Xia Chen, Da Yi, Hui Shi, Yue Sun, Honglei Liu, Xiaobing Cheng, Yutong Su, Junna Ye, Huihui Chi, Zhuochao Zhou, Tingting Liu, Wei Du, Yue Zhao, Chengde Yang, Qiongyi Hu, Jialin Teng","doi":"10.1007/s10067-025-07671-x","DOIUrl":"https://doi.org/10.1007/s10067-025-07671-x","url":null,"abstract":"<p><strong>Objectives: </strong>Pulmonary hemorrhage (PH) represents a rare complication in systemic lupus erythematosus (SLE). In this study, we conducted a thorough investigation into the clinical features, diagnosis, treatment modalities, and outcomes of patients with SLE-associated PH at our medical center. Additionally, a comparative analysis of clinical and laboratory parameters before and after rituximab therapy were performed to assess its efficacy in the management of SLE-associated PH.</p><p><strong>Methods: </strong>Seven SLE-associated PH patients received rituximab therapy at Ruijin Hospital from October 2016 to March 2025 were retrospectively reviewed. The clinical manifestations, laboratory tests, chest CT images before and after treatment were recorded.</p><p><strong>Results: </strong>Among the patients, five were female and two were male, with a median age at diagnosis of 29 years (IQR: 23-64 years). All patients manifested dyspnea, with hemoptysis occurring in 63.5% of cases. The respiratory manifestations of all seven enrolled patients quickly achieved complete recovery and radiographic scan revealed the reduction or complete disappearance of pulmonary infiltrates. The decreased hemoglobin levels quickly rebounded. There was a decrease in the levels of IgG, IgA and IgM concurrently with an increase in C3 and C4. The levels of anti-dsDNA antibodies and positive anti-phospholipid antibodies, SLEDAI-2 K Score showed significant decrease after rituximab treatment. Additionally, the proportion of CD19⁺ and CD20⁺ lymphocytes rapidly reduced after one month of rituximab treatment. The median follow-up duration after rituximab treatment was 23 months (IQR: 17-32 months). All seven patients achieved remission and did not experience a relapse of PH and SLE.</p><p><strong>Conclusion: </strong>Careful consideration is needed in diagnosing SLE-associated PH in young female patients presenting with dyspnea, severe anemia, and pulmonary infiltration, even in the absence of hemoptysis. Rituximab likely demonstrates effectiveness in treating SLE-associated PH, indicating rituximab should be regarded as an alternative treatment for these patients. Key Points • Pulmonary hemorrhage (PH) is a rare yet severe complication of SLE. The treatment for PH in SLE remains uncertain, and evidence for rituximab use is limited to a few case reports. • This study presents a relatively large patient cohort, demonstrating that rituximab effectively resolves clinical symptoms and improves laboratory parameters. • Rituximab, by targeting B cells, may be a promising therapeutic option for SLE-associated PH.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144991583","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":"Long-term renal outcomes of systemic lupus erythematosus in a Taiwanese population: a single-center retrospective study.","authors":"Chao-Han Liu, Chew-Teng Kor, Kai-Hung Hsiao, Ya-Chih Tien","doi":"10.1007/s10067-025-07618-2","DOIUrl":"https://doi.org/10.1007/s10067-025-07618-2","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the long-term outcome of patients with systemic lupus erythematosus (SLE) who presented with renal involvement in a Taiwanese population.</p><p><strong>Methods: </strong>Data of patients diagnosed with SLE at Changhua Christian Hospital Clinical Research Database from January 2010 until December 2021 were retrospectively reviewed. Participants were categorized according to the presence of renal involvement, defined as a high protein-to-creatinine ratio or positive histopathology of kidney biopsy. Incidence rates and risk factors of chronic kidney disease (CKD) and end-stage renal disease (ESRD) were analyzed.</p><p><strong>Results: </strong>A total of 2184 eligible patients with SLE were enrolled, of which 293 had renal involvement. The risk of CKD/ESRD development was higher among participants with renal involvement compared with those without renal involvement. The median times to development of CKD and ESRD were 2.03 years (IQR: 0.13-6.33) and 7.34 years (IQR: 3.13-10.41), respectively, among renal involvement participants. The cumulative incidence rates of CKD were 46.6%, 54.5%, and 63% at 1, 2, and 5 years after renal involvement, respectively, and those of ESRD were 4%, 7%, and 11%, respectively. In the subgroups of two definitions (PCR- or biopsy-defined renal involvement) for renal involvement, the incidence rate of developing CKD/ESRD was not significantly different between the groups. Multivariate analysis revealed that factors associated with CKD development were renal involvement, age, hypertension, and serositis.</p><p><strong>Conclusion: </strong>Among the Taiwanese population, compared with patients without renal involvement, patients with renal involvement were at a higher risk of developing CKD, and the 5-year incidence rate of ESRD is 11% in this study. Key Points • Among the patients with systemic lupus erythematosus in the Taiwanese population, patients with renal involvement were at a higher risk of developing CKD and ESRD. • The median times-to-development of CKD and ESRD were 2.03 (IQR: 0.13-6.33) years and 7.34 (IQR: 3.13-10.41) years, respectively, among renal involvement participants, whilst the cumulative incidence rates of CKD and ESRD at 5 years after renal involvement were 63% and 11%, respectively. • Subgroup analyses to investigate patients with PCR- and biopsy-defined renal involvement were additionally performed in this study, revealing similar incidence rates of CKD and ESRD in both subgroups.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144945456","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":"Leucine-rich alpha-2-glycoprotein 1 (LRG1) decrement during biologics therapy and its correlation with disease features and treatment outcomes in rheumatoid arthritis patients.","authors":"Liang Zou, Qiuyu Fan, Ya Liu, Hao He, Chao Jia","doi":"10.1007/s10067-025-07545-2","DOIUrl":"10.1007/s10067-025-07545-2","url":null,"abstract":"<p><strong>Objectives: </strong>Leucine-rich alpha-2-glycoprotein 1 (LRG1) was previously reported to regulate inflammation and arthritis progression. This study aimed to investigate the correlation of serum LRG1 level with disease features and response to biologics in rheumatoid arthritis (RA) patients.</p><p><strong>Methods: </strong>Seventy-eight RA patients who underwent biologics treatment were analyzed. Serum LRG1 level was detected by enzyme-linked immunosorbent assay at baseline (before biologics were initiated) and at weeks 6 and 12. Treatment response, low disease activity (LDA), and remission were analyzed on the basis of disease activity score in 28 joints. Moreover, serum LRG1 level in another 20 health controls was also analyzed.</p><p><strong>Results: </strong>LRG1 was greater in RA patients than in health controls (46.3 versus 28.6 µg/mL, P < 0.001), with an area under the curve of 0.795 for differentiating them according to the receiver operator characteristic curve. By correlation analysis, LRG1 was correlated with a greater body mass index (P = 0.007) and C-reactive protein level (P = 0.013) in RA patients and tended to be associated with swollen joint count but was not statistically significant (P = 0.052). Furthermore, LRG1 decreased from baseline to week 12 after biologics treatment in RA patients (P < 0.001). However, baseline LRG1 was not correlated with treatment response (P = 0.987), LDA (P = 0.405), or remission (P = 0.763) in RA patients. A decrease in LRG1 at week 12 (P = 0.028) was related to response achievement, and a decrease in LRG1 at week 6 (P = 0.047) and week 12 (P = 0.019) was related to LDA achievement.</p><p><strong>Conclusion: </strong>LRG1 may aid in RA disease supervision, but further validation is needed. Key Points • LRG1 level can distinguish RA patients from health controls with a high AUC at 0.795. • LRG1 level is correlated with higher BMI and CRP level, and tends to be related to elevated SJC in RA patients. • LRG1 level after treatment is correlated with clinical response and LDA to biologics in RA patients, while its level before treatment fails to do so. • Collectively, LRG1 level shows a potential to be a biomarker for RA disease supervision.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"3391-3398"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12397180/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144642014","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgG4-related arthritis revealed by synovial biopsy: a case-based review.","authors":"Alessandra Rai, Giorgia Citriniti, Marianna Oliva, Giuseppe Germanò, Riccardo Valli, Alberto Cavazza, Carlo Salvarani, Niccolò Possemato","doi":"10.1007/s10067-025-07584-9","DOIUrl":"10.1007/s10067-025-07584-9","url":null,"abstract":"<p><strong>Introduction: </strong>IgG4-related disease (IgG4-RD) is a systemic autoimmune condition characterized by fibroinflammatory lesions, potentially occurring at any anatomical site. Typical histopathological features include dense lymphoplasmacytic infiltrates rich in IgG4 + plasma cells, storiform-pattern fibrosis, and obliterative phlebitis. Diagnosing IgG4-RD remains challenging due to its heterogeneous features. Here, we report a rare case of IgG4-RD diagnosed by a synovial biopsy of the wrist.</p><p><strong>Case report: </strong>An 80-year-old man presented with relapsing right wrist arthritis. Laboratory tests showed elevated erythrocyte sedimentation rate and C-reactive protein, while immunological profiles remained negative. Imaging showed severe erosive arthritis of the carpal bones. A synovial biopsy revealed low-grade synovitis (grade 2 according to Krenn score), with histological examination showing significant infiltration of IgG4 + plasma cells (up to 40 per high-power field, with an IgG4/IgG ratio of 40%). 18-Fluorodeoxyglucose-positron emission tomography (PET) showed increased uptake in lymph nodes, with excisional biopsy revealing reactive changes along with a focal increase in IgG4-positive plasma cells (up to 70 per high-power field) and an IgG4/IgG ratio of 50%. Consequently, IgG4-RD was diagnosed, and corticosteroid therapy was initiated, resulting in clinical improvement and reduced IgG4 levels.</p><p><strong>Discussion: </strong>This case illustrates a rare case of IgG4-RD involving joints and lymph nodes, diagnosed by synovial biopsy. The presence of IgG4-positive plasma cells in the joint and lymph nodes strongly supported the diagnosis of IgG4-RD. Synovial biopsy can play a significant role in the diagnostic work-up of arthritis, particularly in cases that are difficult to categorize. Further studies are needed to better characterize IgG4-related arthritis.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"3765-3769"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144706594","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}