Clinical Rheumatology最新文献

{"title":"Transcriptomic sequencing of multiple salivary glands combined with bioinformatics analysis reveals key genes in primary Sjögren's syndrome.","authors":"Chenchen Wang, Hongmin Hu, Yinyue Xu, Shasha Wang","doi":"10.1007/s10067-025-07428-6","DOIUrl":"https://doi.org/10.1007/s10067-025-07428-6","url":null,"abstract":"<p><strong>Objective: </strong>Reveal key genes involved in the pathogenesis of Primary Sjögren's Syndrome (pSS) and identify new potential biomarkers and therapeutic targets.</p><p><strong>Methods: </strong>mRNA transcriptome data from pSS patients'and healthy controls'parotid and minor salivary glands were collected from the Gene Expression Omnibus (GEO) database. mRNA sequencing was performed on pSS mouse model submandibular glands. Differentially expressed genes (DEGs) were identified and core genes were screened using protein-protein interaction (PPI)networks. Validation was done through Gene Ontology (GO),Kyoto Encyclopedia of Genes and Genomes (KEGG), immune cell infiltration, heatmap, and Receiver Operating Characteristic (ROC) curve analyses, followed by external validation. Finally, review the clinical studies of drugs targeting these genes.</p><p><strong>Results: </strong>A total of 113 DEGs were identified, yielding 15core DEGs CD8 A, LCK, SYK, CD2, CD247, CD3D, LCP2, CD3G, CCR7, ITK, CXCR4, B2M, CXCL10, CXCL13, and CXCL9.These core genes were enriched in antigen receptor-mediated and T cell receptor signaling pathways, as well as in the chemokine signaling pathway. Immunocell infiltration analysis revealed that, except for B2M, the expression of other core genes is correlated with the proportion of immune cells. Genes like, CXCL13, CXCL9, CXCR4,CD2,CCR7,and ITK exhibited high diagnostic accuracy for distinguish in pSS patients. Core DEGs such as LCK, SYK, LCP2, and ITK was validated in salivary gland data from pSS patients and mouse models. Drugs targeting LCK, SYK, ITK, and other core genes, with their clinical status, were identified.</p><p><strong>Conclusion: </strong>This study identified key genes in pSS, providing novelinsights into pathogenesis, promising biomarkers, and potential therapeutictargets. Key Points • mRNA transcriptomic sequencing was conducted on submandibular gland specimens from NOD mice simulating pSS and normal mice. • Commonly dysregulated core genes were identified across the minor and parotid salivary glands of pSS patients and healthy controls, as well as in the submandibular glands of NOD and normal mice. • ROC analysis was employed to evaluate their predictive value in the diagnosis of pSS.Genes such as CXCL13, CXCL9, CXCR4, CD2, CCR7, and ITK exhibited high diagnostic accuracy for distinguishing pSS patients. • Genes such as LCK, SYK, and ITK have been validated through external verification and qPCR, and have been identified as targets for clinical drugs.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143971424","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":"Erysipelas-like erythema on elbow in a child with familial Mediterranean fever: an unexpected localization.","authors":"Selçuk Yüksel, Gülten Yüksel","doi":"10.1007/s10067-025-07432-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07432-w","url":null,"abstract":"","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143981941","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 the genetic components and multi-omics sources of inflammatory bowel disease from the perspective of autoimmune disorders.","authors":"Zhonghai Wang, Xin Chen, Quan-Bo Zhang, Han Wang","doi":"10.1007/s10067-025-07422-y","DOIUrl":"https://doi.org/10.1007/s10067-025-07422-y","url":null,"abstract":"<p><strong>Background: </strong>Inflammatory bowel disease (IBD) and autoimmune disorders result from immune system dysregulation. However, the genetic overlap between them remains unclear. Our study aimed to investigate the genetic mechanisms and structure of IBD from the perspective of autoimmune disorders.</p><p><strong>Methods: </strong>The genetic correlation (rg) between traits can provide valuable information about the shared underlying biological mechanisms. Utilizing summary statistics from genome-wide association studies, we delved into the genetic correlation, shared inheritance, and potential causality of IBD (N = 34,652) with autoimmune disorders (N = 1,755,610). We performed transcriptomics at the gene level, multi-marker analyses of genome annotations, and enrichment analyses of biological pathways to highlight shared and diverse perspectives.</p><p><strong>Results: </strong>There were significant genetic correlations between IBD and ankylosing spondylitis (rg = 0.327), rheumatoid arthritis (rg = 0.242), type 1 diabetes (rg = - 0.061), psoriasis (rg = 0.246), and ankylosing spondylitis (rg = 0.308). We identified 110 unique regions (including 5p33.3, 10q25.3, and 22q13.31) after a consistent study at the gene levels. By implementing transcriptomics techniques, we discovered potential common biological mechanisms in several tissues, including blood, spleen, thyroid, and pancreas, revealing potential common biological mechanisms involving lincRNA, protein-coding, and pseudogenes.</p><p><strong>Conclusion: </strong>Our study demonstrated hypothesized pleiotropic genomic regions that provide important clues to delve into the genetic basis of IBD and autoimmune disorders on the basis of multi-omics. Moreover, we have identified shared pathogenic processes and potential common therapeutic targets among these diseases. Key Points • The finding provides a new perspective on the genetic basis of inflammatory bowel disease and autoimmune disease across multi-omics platforms. • Fine mapping of functional summary-based imputation and causal genomes has identified hypothesized pleiotropic genomic regions. • The identified genes and pathways may offer innovative targets for the prevention of immune-related diseases.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143957237","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 features and risk factors of flare in anti-synthetase syndrome.","authors":"Anna Hasegawa, Kazuhiro Kurasawa, Ryota Koike, Rika Sato, Azusa Kikuchi, Sara Komatsu, Yusuke Sakaue, Yuki Aizawa, Aya Shimizu, Yuhi Yoshida, Tomoka Hiyama, Tomoyuki Miyao, Ayae Tanaka, Satoko Arai, Reika Maezawa, Masafumi Arima, Kei Ikeda","doi":"10.1007/s10067-025-07398-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07398-9","url":null,"abstract":"<p><strong>Objectives: </strong>To clarify clinical features and risk factors of flares in anti-synthetase syndrome (ASS).</p><p><strong>Methods: </strong>We retrospectively identified consecutive patients with ASS by reviewing medical records. Clinical, laboratory, and imaging data were obtained. Patients were considered to have a flare when treatment was intensified for myositis and/or ILD with an increased dose of glucocorticoid (GC) and/or addition of a new immunosuppressant (IS).</p><p><strong>Results: </strong>A total of 51 subjects were included. All 51 patients had ILD, 38 patients had myositis, and 39 patients had skin rash. Anti-aminoacyl-tRNA synthetase (ARS) antibody subtypes were anti-Jo-1 in 23 (45%), anti-EJ in 13 (25%), anti-PL-7 in 8 (16%), and anti-PL-12 in 7 (14%). As initial therapy, half of the patients were treated with GCs alone, while the rest were treated with a combination of GCs and ISs during observation. Flares occurred in 30 (58%) mostly when the prednisolone dose was tapered to 11 mg/day. Patients with anti-EJ or anti-Jo-1 frequently flared. The use of calcineurin inhibitors (CNIs) was significantly associated with less flares of myositis but not with those of ILD. In multivariate analyses, anti-ARS subtype and Gottron's sign were identified as independent risk factors for overall flare.</p><p><strong>Conclusion: </strong>The majority of ASS patients experienced a flare, which frequently occurred when the GC dose was tapered to 11 mg of prednisolone. The presence of anti-EJ/Jo-1 antibody and Gottron's sign were identified as independent risk factors for flare. In addition, the use of CNIs tended to associate with less flares of myositis. Key Points • The majority of patients with anti-synthetase syndrome experience a flare when glucocorticoids are tapered close to 10 mg of prednisolone. • The presence of anti-EJ/Jo-1 antibody and Gottron's sign are risk factors for flares in patients with anti-synthetase syndrome. • The use of calcineurin inhibitors may prevent flares of myositis in patients with anti-synthetase syndrome.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143971243","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":"CD3 + CD4 + T cells counts reflect the severity and prognosis of invasive pulmonary aspergillosis in patients with connective tissue disease-associated interstitial lung disease.","authors":"Shenyun Shi, Ruyi Zou, Rui Li, Tingting Zhao, Chao Wu, Yonglong Xiao, Xuebing Feng, Lulu Chen","doi":"10.1007/s10067-025-07425-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07425-9","url":null,"abstract":"<p><strong>Objectives: </strong>Invasive pulmonary aspergillosis (IPA) is a potentially fatal complication in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The aim of this study is to investigate the clinical significance of CD3 + CD4 + T cells counts in CTD-ILD with IPA patients.</p><p><strong>Methods: </strong>This retrospective study included 152 CTD-ILD patients admitted to a single center in China between January 2018 and June 2020. A total of 54 CTD-ILD patients with IPA were assigned to the CTD-ILD with IPA group, while 98 uninfected CTD-ILD patients were assigned to the control group. Serum CD3 + CD4 + T cells counts were compared between the above-mentioned two groups, and the correlations between CD3 + CD4 + T cells counts and the clinical features, mortality of CTD-ILD with IPA were also evaluated.</p><p><strong>Results: </strong>CTD-ILD patients with IPA had significantly lower CD3 + CD4 + T cells counts than those with CTD-ILD without IPA (P < 0.001). The area under the receiver operating characteristic curve (AUROC) of discriminating CTD-ILD with IPA from CTD-ILD without IPA was 0.800 (95% CI, 0.722-0.878, P < 0.001). Correlation analyses showed that serum CD3 + CD4 + T cells counts were positively correlated with PaO2/FiO2 ratio(r = 0.317, P = 0.034) and negatively correlated with C reactive protein (CRP) (r = - 0.358, P = 0.009), erythrocyte sedimentation rate (ESR) (r = - 0.346, P = 0.014), and lactate dehydrogenase (LDH) (r = - 0.306, P = 0.026). In addition, 30 decedents with CTD-ILD infected IPA exhibited lower values of CD3 + CD4 + T cells compared with 24 survivors (P = 0.041). Furthermore, CD3 + CD4 + T cells counts were a prognostic factor and also associated with a higher mortality rate (log-rank test, P = 0.003).</p><p><strong>Conclusion: </strong>CD3 + CD4 + T cells counts could be a useful serum indicator associated with occurrence of IPA in CTD-ILD. Moreover, decreased CD3 + CD4 + T cells counts were associated with a poor survival of IPA in CTD-ILD patients. Key Points • CTD-ILD patients with IPA had significantly lower CD3+CD4+T cells counts than those with CTD-ILD without IPA. • Correlation analyses showed that serum CD3+CD4+T cells counts were positively correlated with PaO2/FiO2 ratio and negatively correlated with C reactive protein (CRP), erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH). • Decreased CD3+CD4+T cells counts were associated with a poor survival of IPA in CTD-ILD patients.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810661","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":"Physical and mental disability is evident 8 years after diagnosis in early rheumatoid arthritis despite contemporary medication and non-pharmacological interventions.","authors":"Ingrid Thyberg, Magnus Husberg, Alf Kastbom","doi":"10.1007/s10067-025-07399-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07399-8","url":null,"abstract":"<p><strong>Introduction: </strong>Early interventions are known to reduce disease activity and physical disability in rheumatoid arthritis (RA), but less is known about mental health, especially in the era of early active pharmacotherapy. Consequently, we compared long-term physical and mental disability in an early RA cohort (1996-1998) with a later cohort (2006-2008).</p><p><strong>Methods: </strong>We compared 320 patients from our project Early Intervention in RA (1996-1998) (TIRA-1) with 463 patients from TIRA-2 (2006-2008). During the 8-year follow-up, pharmacotherapy and multi-professional interventions were offered according to guidelines. Disease Activity Score (DAS28), prescribed disease-modifying antirheumatic drugs (DMARDs), Health Assessment Questionnaire (HAQ), and Short Form Health Survey (SF-36) were registered yearly.</p><p><strong>Results: </strong>Significantly more patients were prescribed DMARDs in TIRA-2 than in TIRA-1, and initial improvements were seen for DAS28 and disability in both cohorts. At follow-up, TIRA-2 patients reported less physical disability (HAQ) and less mental disability (SF-36) than TIRA-1 patients. Despite improvements, 32% of the women and 21% of the men in the TIRA-2 cohort reported considerable disability (HAQ ≥ 1) at the 8-year follow-up.</p><p><strong>Conclusions: </strong>Despite improvements in our contemporarily treated TIRA-2 cohort, physical and mental disability was evident 8 years after diagnosis, especially among women. These results suggest a forthcoming need for person-centered non-pharmacological rehabilitation programs to optimize physical and mental function and to improve participation in daily life in RA. Also, the results highlight the need for developing new interventions directed at reducing disability. Key Points • Physical and mental disability is still considerable in contemporarily treated RA. • Interventions specifically aimed to reduce these disabilities need to be further developed. • Patients with severe disability need to be identified in clinical settings and offered person-centered rehabilitation.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810665","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":"MTX-induced gastrointestinal reactions in RA: Prevotella enrichment, gut dysbiosis, and PI3K/Akt/Ras/AMPK pathways.","authors":"Ruixue Duo, Yining Wang, Quanzhi Ma, Xiaoyuan Wang, Yan Zhang, Haili Shen","doi":"10.1007/s10067-025-07406-y","DOIUrl":"https://doi.org/10.1007/s10067-025-07406-y","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the role of gut microbiota in methotrexate (MTX)-induced gastrointestinal reactions (MRGR) in patients with rheumatoid arthritis (RA).</p><p><strong>Methods: </strong>As a prospective, single-center, convenience sampling study, stool samples were obtained from 28 RA patients (male: female = 10:18) at Lanzhou University Second Hospital who were undergoing MTX treatment for analysis of their gut microbiota using 16S rRNA gene sequencing. Clinical disease activity (CDAI) and MRGR were assessed after two months of MTX therapy. All data collection periods exceeded one year. Intestinal germ-free mice, generated through antibiotic treatment, received fecal microbiota transplantation (FMT) from the patients, followed by varying doses of MTX to observe MRGR. Intestinal transcriptomics and markers related to intestinal barrier function were subsequently examined.</p><p><strong>Results: </strong>Females (84.6%) and high disease activity (CDAI scores, 39.6 ± 11.2 vs 26.3 ± 9.2) were prone to have MRGR in RA patients. Patients with MRGR (PT-GR) showed lower gut microbial diversity versus non-MRGR (PT-noGR). Prevotella abundance, positively correlated with CDAI and MRGR (p < 0.05), was elevated in PT-GR. Administering 10 mg/kg MTX to mice caused intestinal damage. FMT-GR-MTX mice exhibited weight loss (95.2%), morphological deterioration (86.4%), and reduced tight junction proteins (Claudin-1:72.4%; ZO-1:81.2%). Transcriptomics linked upregulated Gβγ/CREB/Atp4b to PI3K/Akt/Ras pathways and downregulated PFK2/PP2 to AMPK signaling in MRGR.</p><p><strong>Conclusion: </strong>Our study identified notable gut microbiota alterations in RA patients prone to MRGR, with changes in intestinal gene expression and reduced intestinal barrier function potentially contributing to MRGR. These findings suggest potential strategies to mitigate MRGR in RA patients undergoing MTX treatment. Key Points • The RA-related MRGR is correlated with the intestinal microbiota. • Females, low gut diversity, and Prevotella enrichment are MRGR risks in RA. • Upregulated DEGs in MRGR linked to PI3K/Akt, Ras pathways. • Downregulated DEGs in MRGR focus on the AMPK pathway.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810663","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":"Hyperuricemia in psoriatic arthritis: clinical correlations and implications.","authors":"Samar AbdAlhamed Tabra, Hany M Aly, Saad Ghanem, Mohammed Hassan Abu-Zaid","doi":"10.1007/s10067-025-07400-4","DOIUrl":"https://doi.org/10.1007/s10067-025-07400-4","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PsA) patients may have elevated serum uric acid levels, and hyperuricemia may impact the degree of inflammation and clinical disease severity.</p><p><strong>Objectives: </strong>comparison between PsA patients with and without hyperuricemia and assessment of the effect of hyperuricemia on clinical presentation, disease activity, disease severity, and associated comorbidities in PsA patients.</p><p><strong>Methods: </strong>76 PsA patients with hyperuricemia and 74 PsA patients with normal uric acid as control were included. Demographic, clinical, comorbidities, and laboratory data were collected. Hyperuricemia threshold ≥ 60 mg/L.</p><p><strong>Results: </strong>There were no significant differences between patients with and without hyperuricemia regarding gender, PsA articular subtype, PASI score, and treatment received, while patients with hyperuricemia were older (40.47 ± 8.53 vs 34.59 ± 7.29, p = 0.0001), had more comorbidity, higher body mass index (BMI) (28.49 ± 2.07 vs 26.91 ± 1.63kg/m2, p = 0.0001), DAPSA score (16.75 ± 7.04 vs 9.32 ± 6.35, p = 0.0001), ESR (34.78 ± 7.12 vs 28.55 ± 8.97, p = 0.0001), CRP (11.42 ± 3.23 vs 8.68 ± 4.04, p = 0.0001), serum cholesterol (220.42 ± 46.83 vs 169.82 ± 37.82, p = 0.0001), and triglycerides (136.47 ± 36.4 vs 104.89 ± 22.15, p = 0.0001), and longer duration of Psoriasis and PsA. The serum uric acid levels were significantly positively correlated with age, duration of Psoriasis, duration of PsA, BMI, CRP, ESR, DAPSA, and PASI score. Multivariate analysis showed that male sex, BMI, and increased disease activity were independent predictors of hyperuricemia in PsA patients.</p><p><strong>Conclusion: </strong>Psoriatic arthritis patients with hyperuricemia have higher age, BMI, disease activity, and more associated comorbidities. In PsA patients, hyperuricemia was associated with male sex, BMI, and increased disease activity, but not associated with PASI score. Key Points • Psoriasis, PsA, and hyperurice s is a bi-centric case-control retrospective of cardiovascular disease. • Male gender, BMI, and increased disease activity were independent predictors of hyperuricemia in PsA patients. • Psoriatic arthritis patients with hyperuricemia have been more associated with comorbidities.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794805","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 relevance of complement levels in assessing the activity of lupus nephritis of different pathological types.","authors":"Weiji Xie, Yixin Zhang, Shiting He, Xuewan Lin, Shuping Zhao, Zeen Xiao, Yimin Zhang","doi":"10.1007/s10067-025-07429-5","DOIUrl":"https://doi.org/10.1007/s10067-025-07429-5","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease that predominantly affects women of childbearing age. Lupus nephritis (LN) is a relatively common and serious complication in clinical patients. The aim of this study was to evaluate the correlation of complement levels and SLEDAI- 2000 (SLEDAI- 2 K) with renal activity in different pathological types of LN.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;A total of 220 patients with SLE and LN were included. Renal active inflammation was calculated using the National Institutes of Health (NIH) Activity Index (AI) . Patients were classified into two groups based on the AI at the time of kidney biopsy: low-to-moderate-activity group with an AI &lt; 10 and high-active group with an AI ≥ 10. Laboratory indicators, including complement levels and the SLEDAI- 2 K, were collected to assess their correlation with renal activity in LN.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The average complement levels in class V LN were higher than that in class III/IV and III/IV + V LN. Serum creatinine and 24-h urine protein were lower than those in class III/IV and III/IV + V LN. Laboratory indicators, including complement levels and SLEDAI- 2 K, shown no correlation with AI in class V LN. Appropriate clinical indicators of AI in patients with class III/IV and III/IV + V LN were further assessed by ROC curves, SLEDAI- 2 K exhibiting the highest performance (AUC 0.757, 95% CI 0.687-0.817), 24-h urine protein (AUC 0.736, 95% CI 0.665-0.798), hemoglobin (AUC 0.726, 95% CI 0.655-0.789), C3 (AUC 0.676, 95% CI 0.603-0.744), serum creatinine (AUC 0.664, 95% CI 0.591-0.733), and C4 (AUC 0.660, 95% CI 0.586-0.729).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;Decreased levels of complement C3 and C4 have limited predictive value as a clinical tool for assessing disease activity in patients with LN, especially in class V LN. SLEDAI- 2 K, a widely used clinical scale, correlates with disease activity in patients with class III/IV, III/IV + V LN. Serum creatinine is a clinical indicator of chronic kidney damage in patients with class III/IV, III/IV + V, and V LN. Key Points Complement levels: can they accurately assess disease activity in lupus nephritis? • Studies investigating the correlation between complement levels and disease activity in patients with SLE and LN yield inconsistent results, and the ambiguity of these findings may stem from factors such as the pathological staging of LN and individual variations in complement levels. Fewer studies in the current research on disease activity in patients with LN will be based on different pathological types of LN, which leads to limitations in the final findings. • This study aimed to analyze the feasibility of complement levels and SLEDAI- 2 K in assessing renal activity in patients with different pathological types of LN. • We assessed disease activity by obtaining AI from renal biopsies in 220 patients with LN and examined their correlation with dise","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794807","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":"Salvage treatment of AOSD-associated macrophage activation syndrome with bariticinib following conventional immunosuppressive therapy.","authors":"Ying Xu, Yufeng Yin, Wenting Li, Jin Zhou, Yan Wang, Yan Huang, TianDan Xiang, Wei Sun, Chuancai Xu, Xiaoping Huang","doi":"10.1007/s10067-025-07352-9","DOIUrl":"10.1007/s10067-025-07352-9","url":null,"abstract":"<p><strong>Objectives: </strong>Adult-onset Still's disease (AOSD)-associated macrophage activation syndrome (MAS) is a highly inflammatory condition. Currently, the main treatment method for AOSD-associated MAS is high-dose glucocorticoid shock therapy, but the effect is not satisfactory, and new therapeutic methods or drugs are urgently needed. The aim of this study was to evaluate the efficacy and safety of salvage baricitinib therapy for AOSD-associated MAS following conventional immunosuppressive therapy.</p><p><strong>Method: </strong>Data from nine patients with MAS treated in the Department of Infection and the Department of Rheumatology, First Affiliated Hospital of Soochow University, from January 2020 to December 2022, were retrospective analysed. Salvage therapy with baricitinib was administered, with an initial dose of 8 mg/day. The patients' clinical symptoms and MAS indices (blood counts and triglyceride, fibrinogen, lactate dehydrogenase, sCD25, and Fer levels) were observed. After a complete response was achieved, the baricitinib dose was gradually reduced to 2-4 mg/day and maintained.</p><p><strong>Results: </strong>The MAS blood test results of all nine patients improved to varying degrees within 1 week post-treatment. The MAS indices gradually returned to normal approximately 8 weeks after discharge; at the 12-month outpatient follow-up, seven patients did not develop MAS again, but two patients (patients 5 and 9) had MAS recurrence. The follow-up period ended with salvage treatment with emapalumab.</p><p><strong>Conclusions: </strong>Baricitinib can effectively inhibit the inflammatory response and improve outcomes in the treatment of MAS secondary to rheumatic immunity-related diseases; moreover, this drug treatment is safe. Our study provides a new strategy for MAS salvage therapy. Key Points • Bariticinib may be a palliative treatment option for recurrent/refractory MAS.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"1705-1712"},"PeriodicalIF":2.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143406185","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}