Rheumatology International最新文献

{"title":"Novel association of chilblains with inflammatory arthritis- series of three cases and review of literature.","authors":"Nilay Joshi, Salamat Ullah, Amit Shah, Shirish Dubey","doi":"10.1007/s00296-025-05840-4","DOIUrl":"https://doi.org/10.1007/s00296-025-05840-4","url":null,"abstract":"<p><p>Chilblains are well circumscribed erythrocyanotic lesions seen usually on limbs distally and triggered by cold and damp conditions; they can be primary or secondary. Association of SLE and chilblains is well established, and there are also reports of associations with vasculitis and antiphospholipid syndrome. There remain considerable gaps in literature about the management of secondary chilblains. Three studies looking at primary chilblains showed some benefit from corticosteroids, but this was inferior to pentoxifylline and tadalafil. There are no trials in secondary chilblains. Chilblains have not been described in the context of inflammatory arthritis- either Rheumatoid Arthritis or seronegative spondyloarthropathy. We report 3 patients with a combination of chilblains, seronegative inflammatory arthritis and phalangeal bone marrow oedema on MRI scans. These patients presented with joint symptoms alongside chilblains with conclusive evidence of inflammatory arthritis. Immunosuppressive treatment resulted in an excellent clinical response. Literature review did not identify any previous descriptions of chilblains together with inflammatory arthritis, whilst bone marrow oedema has primarily been described in children together with chilblains. This case series provides some support for managing secondary chilblains through immunosuppression such as corticosteroids and disease modifying drugs.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"85"},"PeriodicalIF":3.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764935","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":"Reevaluating fibromyalgia diagnosis: a proposal to integrate deep tendon reflex responses into current criteria.","authors":"Ilke Coskun Benlidayi, Ceren Ornek, Volkan Deniz, Aylin Sariyildiz","doi":"10.1007/s00296-025-05846-y","DOIUrl":"https://doi.org/10.1007/s00296-025-05846-y","url":null,"abstract":"<p><p>Fibromyalgia is a complex condition characterized by widespread pain, fatigue, and various other symptoms. The symptoms of fibromyalgia overlap with numerous other disorders (e.g., infections, chronic fatigue syndrome), which makes diagnosis challenging. Existing diagnostic criteria for fibromyalgia rely particularly on subjective patient reports. Such a limitation may lead to both missed diagnoses and potential overdiagnoses. Recent research has identified significantly increased deep tendon reflex (DTR) responses in patients with fibromyalgia. The results also demonstrated the potential for DTR examination to help with the diagnostic process, particularly with ruling out fibromyalgia. The potential underlying mechanisms behind the increased DTR responses in fibromyalgia are central nervous system dysregulation, altered muscular properties, autonomic nervous system dysfunction, and accompanying conditions such as hypomagnesemia and anxiety. By integrating DTR responses into current diagnostic criteria sets, physicians may more effectively differentiate fibromyalgia from other conditions and avoid the pitfalls of misdiagnosis, as well as overdiagnosis. The use of DTR testing in the diagnostic evaluation of fibromyalgia shows promise. Yet, it has both advantages and limitations. The potential benefits of this approach include improved diagnostic accuracy, but challenges remain in its low specificity. This means that hyperreflexia testing alone is not definitive in diagnosing fibromyalgia. Nonetheless, given the high sensitivity, a decreased DTR response could still contribute to ruling out fibromyalgia.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"84"},"PeriodicalIF":3.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764937","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":"Ηidradenitis suppurativa associated with autoimmunity in children and adolescents; a case-based review.","authors":"Maria M Berikopoulou, Argyro Konstantopoulou, Sotirios Argyrakis, Maria Theochari, Panagiota Drosatou, Anna Messaritaki, Dimitra Dimopoulou","doi":"10.1007/s00296-025-05828-0","DOIUrl":"https://doi.org/10.1007/s00296-025-05828-0","url":null,"abstract":"<p><p>Hidradenitis suppurativa (HS) is an uncommon chronic inflammatory disease in children, characterized by painful abscesses and lesions. It has been associated with various autoimmune conditions, including inflammatory bowel diseases (IBDs) and rheumatologic disorders. We present a 13-year-old female with Hurley stage III HS, who developed macrophage activation syndrome (MAS). Clinical presentation and laboratory findings led to a diagnosis of systemic lupus erythematosus (SLE). Treatment with immunosuppressive therapy resulted in symptom resolution and clinical improvement. In addition, a case-based review was conducted to evaluate the patterns, clinical features and possible underlying mechanisms in the pediatric patients with HS and autoimmune diseases. Twenty pediatric cases were included with a median HS onset age of 15 years (IQR: 4 years). Among these, 55% were diagnosed with IBDs, followed by autoimmune arthritis and psoriasis (10% each). Treatment was individualized, with 95% receiving biologic agent and 55% receiving antimicrobials. This case and case-based review of literature highlight the potential overlap between HS and autoimmunity, especially SLE, in the pediatric population and emphasize the importance of proactive monitoring for coexisting autoimmune diseases in patients with HS, in order to facilitate early detection and effective management.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"83"},"PeriodicalIF":3.2,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143736206","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 incidence of hypersensitivity to non-steroid anti-inflammatory drugs in the group of patients with rheumatoid musculoskeletal disorders: the cross-sectional study.","authors":"Olga Brzezińska, Agnieszka Cieplucha, Krystian Słodkowski, Anna Lewandowska-Polak, Joanna Makowska","doi":"10.1007/s00296-025-05834-2","DOIUrl":"10.1007/s00296-025-05834-2","url":null,"abstract":"<p><p>Non-steroidal anti-inflammatory drugs (NSAIDs) are widely used in patients with musculoskeletal disorders. However, hypersensitivity reactions to NSAIDs represent a significant clinical issue.This study aimed to assess the incidence of NSAID hypersensitivity in patients with musculoskeletal disorders and to identify associated risk factors.A total of 343 patients (aged 18-88) with chronic or recurrent musculoskeletal pain were enrolled. Participants completed a questionnaire regarding sociodemographic data, comorbidities, NSAID intake, and side effects. Patients with clinical features of NSAID hypersensitivity were identified and analysis of hypersensitivity risk factors was performed.The study included 343 patients suffering from rheumatic diseases, the most common of which were rheumatoid arthritis (45%), osteoarthritis (32%) and seronegative spondyloarthropathies (11%). However, 24.78% met the criteria for NSAID hypersensitivity. The most common reactions were NSAID-induced urticaria/angioedema (25.88%), delayed hypersensitivity, and NSAID-exacerbated respiratory disease (NERD). The symptoms were most frequently associated with ketoprofen (29%) and diclofenac (25%). Key risk factors for hypersensitivity included daily NSAID intake, asthma, chronic urticaria, smoking, aspirin use at cardiological doses, and a history of allergic reactions. Patients with hypersensitivity had significantly more chronic diseases compared to non-hypersensitive individuals (2.73 ± 2.29 vs. 1.68 ± 1.92; p = 0.0437). A logistic regression model demonstrated a sensitivity of 84.7% and specificity of 72.1% for identifying high-risk patients in teaching and respectively 80.0% and 71.7% in validation group.Identifying high-risk individuals can optimize pain management strategies and reduce adverse outcomes. Selective COX-2 inhibitors, paracetamol, or tramadol may be safer alternatives for patients at risk of hypersensitivity. Further studies are required to validate these findings in larger cohorts.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"82"},"PeriodicalIF":3.2,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143721372","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":"Prevalence of psychiatric and sleep disorders and their impact on quality of life in children with hypermobile Ehlers-Danlos syndrome: an observational study.","authors":"Amanda K Hertel, Jordan T Jones, Ashley Lytch, Emily Cramer, Ariana Schroeder, William R Black","doi":"10.1007/s00296-025-05836-0","DOIUrl":"10.1007/s00296-025-05836-0","url":null,"abstract":"<p><p>Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common connective tissue disorder. However, few studies exist on psychiatric and sleep disorders in pediatric patients with hEDS. This study aims to describe psychiatric and sleep disorders and evaluate their impact on health-related quality of life (HRQoL) in pediatric patients with hEDS. As part of a longitudinal study, a convenience sample of 123 pediatric patients with hEDS, were recruited at a hEDS multidisciplinary clinic in sequential order over a seven-month period. Patient-reported outcomes were completed (Patient Reported Outcomes Measurement Information System Pediatric Profile Version 2 [PROMIS], Generalized Anxiety Disorder-7 [GAD-7], Adolescent Sleep Wake Scale [ASWS], and Pediatric Quality of Life Inventory Rheumatology Module [PEDS-QL Rheum]). The mean age was 15.8 years (SD = 2.7), the majority were female (92%) and Caucasian (92%). Most (86%) had at least one psychiatric diagnosis, with anxiety (80%) the most common, followed by depression (42%). Many (42%) also reported poor sleep. Correlations were seen between the GAD-7, PROMIS pain and HRQoL. Worse anxiety and depression were correlated with more sleep problems. Depression, GAD-7, and ASWS were also correlated with worse HRQoL. Performing regression analyses found anxiety and pain to be most predictive of HRQoL. Psychiatric and sleep disorders are prevalent in pediatric patients with hEDS and impact HRQoL negatively. Clinical focus on the anxiety and pain are important due to their impact on HRQoL.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"81"},"PeriodicalIF":3.2,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11937059/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710481","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":"Controlling nutritional status score as a sensitive instrument for malnutrition screening in systemic sclerosis - a retrospective study.","authors":"Jakub Gajdecki, Bartosz Stępień, Kinga Gajdecka, Olga Brzezińska, Aleksandra Nadel, Joanna Makowska, Aleksandra Opinc-Rosiak","doi":"10.1007/s00296-025-05830-6","DOIUrl":"https://doi.org/10.1007/s00296-025-05830-6","url":null,"abstract":"<p><p>Despite the high prevalence of malnutrition in Systemic Sclerosis (SSc), it often remains overlooked in daily routine. The study aimed to assess the nutritional status of SSc patients using two different tools, concerning their clinical presentation and laboratory results. This retrospective study included 44 SSc patients. We assessed malnutrition with the Malnutrition Universal Screening Tool (MUST) and the Controlling Nutritional Status (CONUT) score. Systemic involvement and laboratory parameters were analysed. Statistical analysis was performed using ANOVA Kruscal-Wallis with post-hoc tests for continuous variables and Chi2 tests for dichotomous variables. The CONUT revealed a substantial proportion of malnourished patients (n = 27; 61,4%), which was higher compared to the assessment with MUST (n = 14; 34,1%). CONUT-undernourished patients had higher C-reactive-protein (28,84 ± 31,72 versus 2,91 ± 2,18, p = 0,0126), higher red-cell-distribution-width (moderate-16,46 ± 2,52 versus normal-13,90 ± 1,03, p = 0,0150), lower hemoglobin (moderate-11,45 ± 2,28 versus normal-13,49 ± 1,28, p = 0,0426), higher N-terminal pro-B-type-natriuretic-peptide (moderate-3790,53 ± 6810,00 versus normal-193,87 ± 265,16, p = 0,0406). However, these observations were not confirmed for MUST-assessed malnutrition. The prevalence of malnutrition in our study was similar to other described cohorts. The CONUT score appeared to be a more sensitive screening tool for malnutrition, however, in patients with cardiological involvement, it may give false-positive results. This study is the first to evaluate SSc patients with the CONUT score, highlighting the need for further research to assess its effectiveness thoroughly.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"80"},"PeriodicalIF":3.2,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700882","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":"YouTube as a source of information for stroke rehabilitation: a cross-sectional analysis of quality and reliability of videos.","authors":"Meirgul I Assylbek, Olena Zimba, Ahmet Akyol, Marlen Yessirkepov, Burhan Fatih Kocyigit","doi":"10.1007/s00296-025-05832-4","DOIUrl":"10.1007/s00296-025-05832-4","url":null,"abstract":"<p><strong>Introduction: </strong>Due to YouTube's meteoric rise in popularity, the quality and reliability of health-related videos on YouTube are being questioned, particularly in specialized fields like stroke rehabilitation. This research aimed to assess the quality and reliability of YouTube videos relevant to stroke rehabilitation.</p><p><strong>Method: </strong>Video listing was conducted on December 17, 2024, using the keywords \"Stroke Rehabilitation\", \"Stroke Physical Therapy\", \"Stroke Neurophysiotherapy\", and \"Stroke Physical Therapy Techniques\" as query terms. A final sample of 72 videos was selected upon completion and evaluated according to inclusion and exclusion criteria. The Global Quality Scale (GQS), Modified DISCERN Questionnaire, JAMA Benchmark Criteria, and Patient Education Materials Assessment Tool for Audio/Visual Materials (PEMAT-A/V) were among the evaluation tools used to analyze each video. Researchers captured the videos' fundamental components and compared the quality classifications.</p><p><strong>Results: </strong>Of the 72 videos examined, 29.2% (n = 21) were categorized as low quality, 20.8% (n = 15) as intermediate level, and 50% (n = 36) as high quality. Videos generated by academic medical centers (77.8%) and nonphysician healthcare professionals (59.4%) were primarily of high quality, while videos from independent users (100%) and TV channels (66.7%) displayed the lowest quality. Significant differences were observed when comparing quality groups based on daily views, likes, and comments (p < 0.05). The lowest scores were detected in the low-quality group. Significant correlations were identified between GQS and other evaluative instruments (p < 0.001), indicating consistency across evaluation frameworks.</p><p><strong>Conclusion: </strong>YouTube possesses considerable potential as an instructional tool for stroke rehabilitation. The inconsistency in video quality underscores the necessity for enhanced content control, editing, and the advocacy of high-quality, evidence-based resources. Promoting collaboration among academics, healthcare professionals, and content producers could augment the platform's instructional efficacy.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"77"},"PeriodicalIF":3.2,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11929695/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693201","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":"Massage therapy in rheumatoid arthritis.","authors":"Umida Khojakulova, Marlen Yessirkepov, Olena Zimba, Yuliya Fedorchenko","doi":"10.1007/s00296-025-05833-3","DOIUrl":"https://doi.org/10.1007/s00296-025-05833-3","url":null,"abstract":"<p><p>Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by joint inflammation, pain, and reduced mobility. Although pharmacological treatments, such as therapies with disease-modifying antirheumatic drugs and biologic agents, have improved disease outcomes, many patients continue to experience residual symptoms that impair their quality of life. This review examines the role of massage therapy as a complementary intervention in RA, focusing on its clinical benefits and underlying mechanisms. A systematic search of Medline/PubMed, Scopus, and the Directory of Open Access Journals (DOAJ) up to February 2025 was conducted using relevant MeSH terms. Evidence from clinical studies suggests that massage modalities-including Swedish massage, aromatherapy massage, and foot reflexology-can reduce pain, improve joint function, and modulate inflammatory markers (e.g., IL-6, TNF-α) and cortisol levels, thereby promoting neuroendocrine balance and lymphatic drainage. Furthermore, massage therapy may offer additional benefits in managing comorbid conditions and enhancing psychological well-being. This review also underscores the pivotal role of nurse-led care in integrating massage therapy into comprehensive treatment plans, despite existing challenges in training and standardization of protocols. Future research with larger sample sizes and longer follow-up periods is essential to establish robust guidelines and optimize the use of massage therapy as an adjunct treatment in RA.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"76"},"PeriodicalIF":3.2,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693199","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":"Lasso-derived model for early prediction of systemic sclerosis based on vasculopathy assessment: a population-based cohort study.","authors":"Cong Lei, Xing Luo, Youqun Zhang, Ying Zhang, Linxuan Pang, Yan Wang, Haoyang Sun, Zhaohui Zheng, Yan Zheng, Ping Zhu","doi":"10.1007/s00296-025-05835-1","DOIUrl":"https://doi.org/10.1007/s00296-025-05835-1","url":null,"abstract":"<p><strong>Objective: </strong>Vasculopathy is a key feature of systemic sclerosis (SSc) and is critical for its diagnosis and prognosis. We aimed to comprehensively analyze vascular lesions in patients with SSc and establish an early diagnostic model based on these lesions.</p><p><strong>Methods: </strong>We recruited 111 participants (45 healthy participants and 66 patients with SSc, mean age 49.75 ± 12.902 years). Age, sex, blood pressure, hand grip strength, skin thickness, proper palmar digital artery vascular index, skin blood flow index, and nailfold microcirculation were compared between the two groups. Applying Lasso regression for variable selection, we further developed a binary logistic regression model to analyze the diagnostic differences in disease occurrence based on the vascular injury status. We assessed the performance of the model using receiver operating characteristic (ROC) and calibration curve analyses to evaluate its diagnostic ability and determine the optimal cutoff value.</p><p><strong>Results: </strong>Using Lasso regression analysis, we identified 10 key variables from 37 microcirculation parameters, including age, left hand grip strength, left peak systolic velocity (PSV), right PSV, right resistance index (RI), ischemic perfusion (IPU), ischemic reperfusion perfusion (IRPU), post-occlusive reactive hyperemia baseline (PORH BL), loop top length, and nailfold video-capillaroscopy (NVC) score. Among these, the NVC score (Cut-off point = 5.35, AUC = 0.845, SEN = 0.74, SPE = 0.87), PSV (Cut-off point = 11.38, AUC = 0.838, SEN = 0.82, SPE = 0.73), IRPU (Cut-off point = 111.3, AUC = 0.831, SEN = 0.61, SPE = 0.91), and Grip (Cut-off point = 22.8, AUC = 0.781, SEN = 0.79, SPE = 0.62) demonstrated high diagnostic value in predicting SSc. The binary logistic regression model based on these variables provides better interpretability for advanced diagnosis of microcirculation multidimensional integration. Compared to the scleroderma pattern model, this model exhibited superior performance, with an area under the curve of 0.929 (95% CI: 0.883-0.974).</p><p><strong>Conclusion: </strong>Our results highlight the key role of the nailfold video-capillaroscopy score, grip strength, and peak systolic flow velocity of the proper palmar digital artery in predicting systemic sclerosis events.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"78"},"PeriodicalIF":3.2,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693196","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":"Altered IgG4 serum levels in VEXAS syndrome - a retrospective monocentric cohort study.","authors":"Sebastian J Saur, Benedikt Obermaier, Reinhild Klein, Matthias Hahn, Falko Fend, Sven Mattern, Joerg C Henes, Ann-Christin Pecher","doi":"10.1007/s00296-025-05824-4","DOIUrl":"10.1007/s00296-025-05824-4","url":null,"abstract":"<p><p>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disorder characterized by somatic mutations in the UBA1 gene in hematopoietic stem cells and associated with diffuse inflammation and myelodysplastic changes amongst others. Due to unspecific symptoms the diagnosis is challenging, and there is an unmet need for clinical markers to select patients for genetic examination. Sera of 9 patients with confirmed VEXAS syndrome were analyzed for immunoglobulin (Ig)G4 levels. Disease parameters and clinical response to therapy were correlated with IgG4 levels. A histopathological examination was performed on the available samples to exclude IgG4-related autoimmune diseases. In this cohort, 44% of our patients showed markedly elevated serum IgG4 levels. We observed a general trend toward a positive correlation between IgG4 levels and inflammatory markers as well as a correlation with clinical response in one patient. Histopathological analysis showed no evidence of IgG4 related disease. IgG4 levels seem to be elevated in a relevant fraction of patients with VEXAS syndrome. In some cases, this might be misinterpreted as IgG4-related disease, a pitfall clinicians should be aware of. Furthermore, our results warrant the further evaluation of a potential correlation of IgG4 levels with disease activity and severity of inflammation. IgG4 serum levels might be useful in the evaluation of the disease course.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"79"},"PeriodicalIF":3.2,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11929712/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693194","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}