Zeitschrift fur Rheumatologie最新文献

{"title":"[Cardiac involvement in idiopathic inflammatory myopathies].","authors":"Tim Wilhelmi, Julia Treiber, Ulf Müller-Ladner, Philipp Klemm","doi":"10.1007/s00393-026-01876-4","DOIUrl":"https://doi.org/10.1007/s00393-026-01876-4","url":null,"abstract":"<p><strong>Background: </strong>Cardiac manifestations in idiopathic inflammatory myopathies (IIM) occur more frequently than they are clinically recognized and are associated with increased morbidity and mortality. In particular, subclinical myocarditis often remains undetected due to nonspecific or absent symptoms.</p><p><strong>Objective: </strong>This review aims to summarize the prevalence, pathophysiology, clinical spectrum, diagnostic strategies and therapeutic options of cardiac involvement in IIM and to provide practical recommendations for rheumatologists.</p><p><strong>Methods: </strong>A scoping review on cardiac manifestations in IIM was performed. A structured literature search was conducted using the PubMed database. The search strategy included combinations of relevant keywords and MeSH terms. Primarily, English language original studies, systematic reviews and current guideline publications related to cardiac involvement in IIM were considered. Studies were selected based on clinical relevance, methodological quality and recency.</p><p><strong>Results: </strong>The reported prevalence of cardiac involvement in IIM ranges from 4-26%, depending on the subtype and diagnostic approach. In addition to clinically overt myocarditis, subclinical abnormalities are common and can be detected in up to 50% of patients using cardiac magnetic resonance imaging (CMRI). Pathophysiological mechanisms include immune-mediated myocardial injury, microvascular dysfunction and fibrotic remodelling. The initial diagnostic work-up should comprise taking a medical history, electrocardiography, measurement of cardiac biomarkers and echocardiography. The use of CMRI represents the diagnostic gold standard and enables sensitive detection of inflammatory and fibrotic myocardial changes. Therapeutically, early initiation of drug treatment, typically high-dose glucocorticoids combined with additional immunomodulating agents, constitutes the cornerstone of management.</p><p><strong>Conclusion: </strong>Cardiac manifestations in IIM are common, prognostically relevant and frequently underestimated in clinical practice. A structured diagnostic approach with early use of CMRI and interdisciplinary management is essential to detect subclinical disease and guide appropriate treatment.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149224916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Osteoporosis in rheumatology-Diagnostics, risk assessment and modern treatment strategies].","authors":"Philipp Klemm, Alexander Pfeil","doi":"10.1007/s00393-026-01885-3","DOIUrl":"https://doi.org/10.1007/s00393-026-01885-3","url":null,"abstract":"<p><p>Inflammatory rheumatic diseases such as rheumatoid arthritis (RA) lead to local periarticular demineralization through localized arthritis in one or more joints; however, the autoimmune-mediated systemic inflammatory process is also a driver of systemic bone loss, osteoporosis, which leads to an increased risk of fractures. The Umbrella Organization Osteology (DVO) guidelines 2023 recommend a risk-adapted approach involving systematic fracture risk assessment, early baseline diagnostics and consistent specific osteoporosis treatment, including antiresorptive and osteoanabolic strategies. For rheumatology practice this means firmly integrating osteological assessment, including fracture risk management as well as pharmacological and nonpharmacological osteological treatment into the structured care of patients with inflammatory rheumatic diseases.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Predictors of willingness for COVID-19 vaccination in patients with inflammatory rheumatic diseases at the beginning of the vaccination rollout].","authors":"Dana Delia, Ulf Müller-Ladner, Jörn Pons-Kühnemann, Matthias Broll, Björn Bühring, Eike Domgörgen, Andreas Dormann, Rebecca Hasseli, Farid I Kandil, Ilka Kraeft, Andreas Krause, Hanns-Martin Lorenz, Nicole Morena, Filiz Özden, Alexander Pfeil, Jutta G Richter, Jasemine Saech, Thomas Schneidereit, Andreas Schlesinger, Christof Specker, Kathrin Storck-Müller, Johannes Strunk, Konstantinos Triantafyllias, Daniel Vagedes, Katharina Vesper, Siegfried Wassenberg, Sami Zeglam, Tim Schmeiser","doi":"10.1007/s00393-026-01879-1","DOIUrl":"https://doi.org/10.1007/s00393-026-01879-1","url":null,"abstract":"<p><strong>Introduction: </strong>In January 2021 the coronavirus disease 2019 (COVID-19) was coupled to high case numbers and mortality worldwide and in Germany. The start of the vaccination campaign was initially characterized by limited availability and uncertainty but rapidly gained momentum. Patients with inflammatory rheumatic diseases (IRD) were prioritized at an early stage due to the high risk for a severe disease trajectory. In this group vaccination proved to be effective and greatly reduced the risk of a severe course. The aim of the study was to ascertain the willingness for vaccination and the influencing factors in patients with IRD in order to optimize vaccination strategies and to reach vulnerable groups more easily.</p><p><strong>Methods: </strong>Between January and April 2021 a nationwide survey was carried out in Germany among N= 3661 patients with IRD and N=381 control patients with other chronic internal diseases. The questionnaire covered willingness to undergo vaccination against COVID-19 and demographic and disease-related variables. For the analysis, \"willing to be vaccinated\" and \"already vaccinated\" were pooled (\"vaccination willingness\") and compared the unwilling. The statistical evaluation was carried out using χ2-tests, odds ratio (OR) analyses and logistic regression to identify independent influencing factors.</p><p><strong>Results: </strong>The proportions of those willing to be vaccinated were comparable in both groups with 90% very high (IRD 89.2% and control group 93.9%). They continually increased in both groups during the observational period and with increasing age. Another strong predictor was a previously carried out simultaneous vaccination for influenza, which was associated with a significantly higher willingness to be vaccinated in both groups.</p><p><strong>Conclusion: </strong>The willingness to be vaccinated against COVID-19 at the time of the vaccination rollout in Germany was high. For patients with IRD, it was predicted by age, previous vaccination against influenza and the timing of vaccination but not the subtype of IRD or the type of immunomodulatory medication. Young patients with IRD in particular should be systematically identified as a risk group with a low willingness to be vaccinated. For this patient group an intensified target group-specific vaccination counselling should be integrated into the clinical care, which specifically addresses suitable information channels for those affected.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149168277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Transition: between recommendations and real-world care : Results from the InfoTrans project].","authors":"Florian Milatz, Susanne Schalm, Kirsten Minden","doi":"10.1007/s00393-026-01881-7","DOIUrl":"https://doi.org/10.1007/s00393-026-01881-7","url":null,"abstract":"<p><strong>Background: </strong>The transition from pediatric to adult rheumatology care represents a particular challenge for young people with juvenile idiopathic arthritis (JIA).</p><p><strong>Objectives: </strong>To investigate the continuity of care, disease burden, transition competence and unmet needs among young adults with JIA.</p><p><strong>Material and methods: </strong>Within the Innovation Fund project InfoTrans, a cross-sectional survey was conducted among- individuals insured with the Barmer health insurance aged 16-25 years with JIA and pseudonymously linked with health insurance claims data. For the present analysis, young adults aged 20 years and older with prior pediatric rheumatology care were included. Data collected comprised current healthcare utilization, patient-reported outcomes, transition competence (Transitions-KompAZ), and psychological well-being (WHO-5).</p><p><strong>Results: </strong>Among 269 surveyed young adults with JIA, 45% were receiving specialist rheumatology care. Participants receiving rheumatology care showed higher transition competence (22.7 vs. 19.0; p < 0.001), had more frequently attended a transition consultation (17% vs. 9%), and were more often aware of the website \"mein-rheuma-wird-erwachsen.de\" (33% vs. 20%; p = 0.013). Despite comparable disease burden, participants without specialist care received DMARD therapy less frequently (18% vs. 63%). Gaps in care were also observed among participants receiving rheumatology care: the average interval between the last pediatric rheumatology and first adult rheumatology visit was 9.9 months. Overall, 35% had not received a medical transfer summary report, more than 40% were dissatisfied with preparation for transfer, and approximately 1 in 5 participants reported relevant depressive symptoms.</p><p><strong>Conclusion: </strong>Relevant discrepancies remain between recommendations for structured transitional care and actual care delivery. Disease-related knowledge appears to be a central factor for continuous follow-up in rheumatology. The findings underline the importance of early patient education and better integration of support services.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149149434","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Mental health-An underestimated problem? : Psychological burden in transitional care: recommendations for rheumatological practice].","authors":"Verena Hofmarcher, G Erbis, L Höfel, C Reiser, J-P Haas","doi":"10.1007/s00393-026-01882-6","DOIUrl":"https://doi.org/10.1007/s00393-026-01882-6","url":null,"abstract":"<p><strong>Background: </strong>The transition of patients from pediatric to adult rheumatology represents a psychosocial process that extends beyond a mere transfer of care. Adolescents and young adults are in a particularly vulnerable developmental phase characterized by substantial disease-related and developmental challenges. Mental health has a major impact on disease outcomes, treatment adherence, self-management, and the overall success of transition. At the same time, this life stage is associated with an increased risk of psychological distress and mental disorders.</p><p><strong>Objective: </strong>This article aims to highlight the importance of mental health in rheumatological transitional care and to derive practical recommendations for the screening and management of psychological distress.</p><p><strong>Methods: </strong>The paper is based on a review of selected literature on mental health comorbidities in pediatric rheumatology and transition care. Clinical experiences and established models of care are additionally considered.</p><p><strong>Results: </strong>Mental health should be regarded as an integral component of rheumatological transitional care. A structured approach is recommended, including the integration of routine low-threshold mental health screening into a staged-care model, and interprofessional collaboration.</p><p><strong>Discussion: </strong>Mental health is a key yet often underrecognized factor in transition care. The systematic integration into routine rheumatology practice is essential. Structured screening, staged-care approaches, and interdisciplinary collaboration can help to close existing gaps in care and improve transition outcomes in a sustainable manner. In addition to interdisciplinary care, sustainable implementation requires adequate funding for standardized transition programs.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149128473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Myositis induced by immune checkpoint inhibitor therapy].","authors":"Karolina Gente, Kastriot Kastrati, Helga Lechner-Radner, Rebecca Hasseli-Fräbel","doi":"10.1007/s00393-026-01878-2","DOIUrl":"https://doi.org/10.1007/s00393-026-01878-2","url":null,"abstract":"<p><strong>Background: </strong>Immune-mediated myositis can be a severe side effect of cancer treatment involving immune checkpoint inhibitors (ICI). As a distinct type of rheumatic immune-related adverse events (irAE), it presents challenges in terms of diagnosis and patient management. The high mortality and the potential for rapid progression necessitates prompt treatment.</p><p><strong>Objective: </strong>This article provides a practice-oriented overview of myositis-induced irAEs, focusing on the diagnostic and treatment management and the prognostic implications.</p><p><strong>Material and methods: </strong>A selective literature review was conducted, covering current studies, registry data and pharmacovigilance data as well as guidelines for recognizing and treating ICI-associated myositis irAEs.</p><p><strong>Results: </strong>Myositis-induced irAEs typically manifest within 1 month of initiating ICI therapy. The incidence is between 0.5% and 1%; however, it is significantly higher with ICI combination therapies. In around half of the cases, myositis is the only irAE manifestation. The number of cases of neuromuscular irAE overlap with myocarditis and/or myasthenia rose from 3.7% before to 8.3% after 2017. Around 20% of cases of isolated myositis require ventilation, compared to 50-80% of cases of overlap syndrome, which is associated with a high mortality of 40%.</p><p><strong>Conclusion: </strong>If a myositis-irAE is suspected, high-dose glucocorticoid therapy should be initiated immediately. The treatment often includes glucocorticoid-sparing medications. For severe cases, abatacept should be considered as a salvage therapy. Early detection and close collaboration between all medical specialty disciplines involved are crucial for the prognosis of patients.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149128442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Rheumatology centers (according to G-BA regulations) as pivotal elements of high-quality integrated acute inpatient care for people with inflammatory rheumatic diseases in Germany].","authors":"Alexander Pfeil, Carina Stammann, Heinz-Jürgen Lakomek","doi":"10.1007/s00393-026-01869-3","DOIUrl":"https://doi.org/10.1007/s00393-026-01869-3","url":null,"abstract":"<p><p>Subsequent to the publication of the Federal Joint Committee (G-BA) regulations in the Federal Gazette in March 2020, G‑BA rheumatology centers have been established in Germany as key facilities for providing high-quality acute inpatient care to people with inflammatory rheumatic diseases. In accordance with the prevailing regulations, by May 2026, 31 facilities in 14 of the 16 federal states had been designated, including 24 G-BA rheumatology centers for adults and 7 G‑BA rheumatology centers for pediatric and adolescent rheumatology.The review article describes the evolution and contemporary state of the G‑BA rheumatology centers in Germany, in conjunction with an exposition of the structural prerequisites, task profiles and functions in the care of inpatients with inflammatory rheumatic diseases at other hospitals or specialized rehabilitation facilities. The emphasis is on networking and quality assurance activities, scientific and guideline-based work, the evaluation of innovative treatment and the role of the centers in continuing medical education as special tasks.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149115527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Recommendations of the German Society for Rheumatology committee for complementary treatment and nutrition on phytotherapy : Supplement on the use of selected phytotherapeutic agents and herbal preparations in rheumatology: extension to include 13 medicinal plants].","authors":"G Keyßer, Oliver Sander, Inna Frohne, Alexander Pfeil, Andreas Michalsen, Olaf Schultz, Monika Reuß-Borst, Olga Seifert","doi":"10.1007/s00393-026-01840-2","DOIUrl":"10.1007/s00393-026-01840-2","url":null,"abstract":"<p><strong>Introduction: </strong>Medicinal plants and their preparations are of great interest to many patients with rheumatic diseases. In 2023 the German Society for Rheumatology and Clinical Immunology (DGRh) Committee for Complementary Medicine and Nutrition (KHE) began reviewing the scientific literature on selected phytotherapeutic agents and herbal preparations and examining their potential applications in rheumatology. Following the publication of the initial results in 2025, the evaluation of 13 additional phytotherapeutic agents took place later that same year.</p><p><strong>Methods: </strong>In an online meeting of the KHE Committee on 7 January 2025, a further list of phytotherapeutic agents frequently used in rheumatology (mostly as self-medication) was compiled. Committee members then conducted literature searches on these herbal preparations and summarized the results according to a predefined matrix. Research was conducted on comfrey, arnica, turmeric, ginger, cat's claw (Uncaria tomentosa), garlic, cabbage leaves, milk thistle, Pycnogenol (extract from the bark of maritime pine), horsetail, devil's claw (Harpagophytum), frankincense (Boswellia) and Wilford's tripter fruit (Tripterygium wilfordii). The results were reviewed using a circulation procedure, a consensus was agreed upon in an online meeting of the KHE committee and finally confirmed by the German Society for Rheumatology and Clinical Immunology (DGRh).</p><p><strong>Results: </strong>For all plant substances investigated, there are reports of anti-inflammatory or immunological effects in vitro and/or in animal models; however, the evidence for a clinically relevant benefit is very limited. None of the investigated preparations can be recommended for use in inflammatory joint diseases. If arnica, comfrey, turmeric, ginger, garlic, cabbage leaves, Pycnogenol, devil's claw or frankincense are taken at the patient's own initiative for degenerative joint diseases, rheumatologists do not need to advise against them, provided a sensible treatment plan is otherwise being followed. Ginger, garlic and turmeric in their natural form can be recommended as foods within the framework of a health-conscious diet.</p><p><strong>Conclusion: </strong>The scientific analysis of a second series of herbal preparations has also shown that the confirmed value of phytotherapy for the field is limited; however, for the rheumatological practice a differentiated consideration is needed for the herbal preparations presented here.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"655-672"},"PeriodicalIF":1.2,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13649835/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148449529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Survey of care situation for patients with systemic lupus erythematosus and systemic sclerosis with pulmonary involvement in Rhineland-Palatinate and Saarland].","authors":"Lennart Haßdenteufel, Kai Jahns, Ciaran Alberti, Andreas Schwarting, Matthias Dreher","doi":"10.1007/s00393-026-01830-4","DOIUrl":"10.1007/s00393-026-01830-4","url":null,"abstract":"<p><strong>Background: </strong>Connective tissue diseases such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) require interdisciplinary management due to their multisystem involvement. Pulmonary manifestations (CTD-ILD) are of particular prognostic relevance. However, real-world data on current healthcare provision remain limited.</p><p><strong>Methods: </strong>A cross-sectional survey (14 modules) was conducted between September 2024 and March 2025, targeting 2173 practices across various specialties in Rhineland-Palatinate and Saarland. Data on healthcare structures, patient numbers, pulmonary involvement, and pharmacological treatment were collected. Analysis was performed descriptively at the provider level.</p><p><strong>Results: </strong>A total of 67 institutions reported on 1569 patients (SLE: n = 859; SSc: n = 421), of whom 286 (≈18%) had pulmonary involvement. Care was predominantly delivered in specialized centers, but with substantial contribution from office-based physicians. Marked differences in treatment strategies were observed: office-based settings more frequently relied on higher-dose glucocorticoids (up to ≈28% > 10 mg/day), whereas specialized centers achieved significant glucocorticoid reduction (≈1.5% > 10 mg/day) through increased use of biologics and disease-modifying therapies, particularly mycophenolate mofetil (MMF). MMF was notably more frequently used in rheumatology-based care compared to other specialties.</p><p><strong>Conclusion: </strong>The findings reveal relevant deficiencies in care, particularly an overreliance on glucocorticoids and underutilization of evidence-based disease-modifying antirheumatic drugs (DMARDs) such as MMF in nonspecialized settings. Contributing factors likely include limited rheumatology expertise and insufficient interdisciplinary coordination. Improving care requires expansion of rheumatologic capacity, earlier specialist referral, and broader implementation of guideline-based, steroid-sparing treatment strategies.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"608-619"},"PeriodicalIF":1.2,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13649884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148206290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Turner syndrome as a state of increased autoimmune susceptibility: evidence from rheumatic diseases.","authors":"Jozélio Freire de Carvalho, Rodrigo Antonio Brandao Neto","doi":"10.1007/s00393-026-01812-6","DOIUrl":"10.1007/s00393-026-01812-6","url":null,"abstract":"<p><p>Turner syndrome (TS) is a chromosomal disorder of females due to the partial or complete absence of an X chromosome. Growing evidence suggests a significant association between TS and autoimmune disorders, especially rheumatic diseases like juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). Recognition of this association is important for early detection, timely intervention, and avoidance of complications. The aim of this narrative review is to analyze the association between TS and rheumatic diseases in terms of epidemiological, clinical, and pathophysiological aspects as well as therapeutic options. The narrative review was performed by combining information from published case reports, case series, and population-based studies reporting on the association of TS with autoimmune rheumatic diseases. Juvenile idiopathic arthritis was the most frequently described rheumatic disease in TS, mostly as oligoarticular but also as severe polyarticular forms. Systemic lupus erythematosus was less common, but most patients had severe systemic disease with lupus nephritis. Other manifestations were psoriatic arthritis; ankylosing spondylitis in adult and pediatric cases; and systemic sclerosis as a very rare exception, likely reflecting the scarcity of reported cases rather than a defined protective effect in TS. A population-based study revealed that women with TS are at a profoundly increased risk of autoimmune rheumatic diseases as compared to the general female population. In conclusion, these findings suggest increased autoimmune susceptibility in Turner syndrome and underscore the need for heightened clinical awareness. Current evidence remains limited, underscoring the need for heightened clinical awareness rather than routine screening strategies.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"642-647"},"PeriodicalIF":1.2,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147677215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}