Zeitschrift fur Rheumatologie最新文献

{"title":"Optimal timing of liver stiffness assessment in methotrexate-treated rheumatoid arthritis patients.","authors":"Maroua Slouma, Wided Lahmar, Meriam Ben Messaoud, Ghanem Mohamed, Imen Gharsallah","doi":"10.1007/s00393-026-01875-5","DOIUrl":"https://doi.org/10.1007/s00393-026-01875-5","url":null,"abstract":"<p><strong>Background: </strong>The cumulative methotrexate (MTX) dose threshold for initiating hepatic fibrosis screening in patients with rheumatoid arthritis (RA) remains undefined. This study aimed to identify this threshold and compare the utility of transient elastography versus the Fibrosis‑4 (FIB-4) index as a screening tool.</p><p><strong>Methods: </strong>We conducted a cross-sectional study of consecutive RA patients receiving MTX who had documented normal hepatic evaluation before treatment initiation. All participants underwent clinical assessment, laboratory testing (liver function tests, complete blood count, inflammatory markers), transient elastography (FibroScan; EchoSens, Paris, France), and FIB‑4 index calculation. Receiver operating characteristic (ROC) curve analysis was performed to determine the optimal cumulative MTX dose cutoff for predicting liver fibrosis (defined as a liver stiffness measurement ≥ 6 kPa). Multivariable logistic regression was used to identify independent predictors of liver fibrosis.</p><p><strong>Results: </strong>We included 73 patients. The median liver stiffness was 4.1 kPa (IQR 3.45-4.95). Nine patients (12.3%) had evidence of liver fibrosis (≥ 6 kPa), of whom four (5.5%) had significant fibrosis (≥ 7.2 kPa). The median FIB‑4 index was 0.87 (IQR 0.61-1.20). Liver stiffness significantly correlated with age (r = 0.236, p = 0.045), cumulative MTX dose (r = 0.297, p = 0.003), MTX treatment duration (r = 0.279, p = 0.006), total bilirubin (r = 0.263, p = 0.025), and direct bilirubin (r = 0.622, p = 0.041). The FIB‑4 index correlated with age (r = 0.613, p  0.001), AST (r = 0.482, p  0.001), and bilirubin levels. A cutoff of 2990  mg cumulative MTX dose can discriminate between patients with normal liver function and those with liver fibrosis (sensitivity 100%, specificity 58%; AUC 0.793). This same cutoff can also discriminate between patients with a normal and those with an abnormal FIB‑4 index, albeit with lower sensitivity (69.5%) and specificity (58%) (AUC 0.587; p = 0.298).</p><p><strong>Conclusion: </strong>A cumulative MTX dose of approximately 3 g represents a practical screening threshold for initiating liver fibrosis evaluation in RA patients. Transient elastography is superior to the FIB‑4 index for this purpose. Screening should be individualized based on metabolic risk factors, and elevated liver stiffness should prompt etiological investigation rather than automatic MTX discontinuation. Prospective studies are needed to validate these findings and assess long-term outcomes.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149081917","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":"[Young adults with autoinflammation-What can rheumatologists expect?]","authors":"Marc-Alexander Oestreich, Jessica Pauz, Mary-Louise Daly, Martin Krusche, Tatjana Welzel","doi":"10.1007/s00393-026-01871-9","DOIUrl":"https://doi.org/10.1007/s00393-026-01871-9","url":null,"abstract":"<p><strong>Background: </strong>Advances in diagnostics, molecular genetics and targeted treatment have optimized the management of patients with autoinflammatory diseases (AID). As increasing numbers of children and adolescents with AID survive into adulthood, the transition from pediatric to adult rheumatology care has become an important component.</p><p><strong>Aim of the study: </strong>Presentation of the specific challenges and requirements of the transition of adolescents with AID from pediatric to adult rheumatology care and summary of current evidence and recommendations.</p><p><strong>Material and methods: </strong>Narrative review of the literature including guidelines, consensus statements, registries, cohort studies and surveys addressing transition.</p><p><strong>Results: </strong>Successful transition of adolescents with AID requires an early structured and multidisciplinary process that addresses medical, psychosocial and educational needs, promotes self-management and empowerment and ensures continuity of specific care. Existing transition recommendations for chronic inflammatory rheumatic diseases are generally applicable to patients with AID; however, several AID-specific features necessitate additional considerations, such as genetic causes, relevant comorbidities and often lifelong treatment strategies involving off-label treatment. The AID-specific recommendations and data on the effectiveness of structured transition programs are so far limited.</p><p><strong>Discussion: </strong>The transition of patients with AIDs from pediatric to adult care is a complex and demanding process. Close collaboration between pediatric and adult rheumatologists as well as patient-centered transition strategies, are crucial to ensure disease control, quality of life and social participation.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149076237","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":"[Revision of the model special training regulations 2026].","authors":"Alexander Pfeil, Anna Voormann, Martin Aringer, Isabell Haase, Ina Kötter, Gabriela Riemekasten, Ulf Wagner, Torsten Witte, Martin Fleck, Christof Specker","doi":"10.1007/s00393-026-01868-4","DOIUrl":"https://doi.org/10.1007/s00393-026-01868-4","url":null,"abstract":"<p><p>The revision of the model special training regulations (Musterweiterbildungsordnung, MWBO), the federal template for training of specialists in internal medicine and rheumatology, occurs in the context of significant structural changes in the German healthcare system. Medical care structures will change, for inpatient care based on the Hospital Care Improvement Act (KHVVG) and the Hospital Reform Adjustment Act (KHAG), for outpatient care based on changes towards the primary care providers and all caregivers will have to adapt to the Health Insurance Premium Stabilization Act (BStabG). At the 130th German Federal Medical Assembly, an amendment was adopted for the MWBO with respect to training periods. The new MWBO maintains the total training period of 72 months for all internal medicine subspecialties but reduces the mandatory inpatient training period in the specialty from 24 to 18 months. In this context, the present article analyzes how the amended MWBO can ensure specialist training in rheumatology that is of high quality and fit for the future.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149064862","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":"[Psoriatic arthritis as MHC-I-associated disease].","authors":"Michaela Köhm, Anke König, Isabel Rzehak, Nadia Ninosu, Magdalena Figat, Frank Behrens","doi":"10.1007/s00393-026-01864-8","DOIUrl":"https://doi.org/10.1007/s00393-026-01864-8","url":null,"abstract":"<p><p>Psoriatic arthritis is a heterogeneous, immune-mediated systemic disease with manifestations affecting joints, entheses and skin. A characteristic feature is the absence of autoantibodies, while mechanisms of innate and tissue-resident immunity predominate. The concept of MHC-I-associated diseases provides an integrative framework for pathogenetic classification. Central elements of the concept include the association with HLA class I alleles, alterations in antigen processing, for example mediated by ERAP1/2, and activation of the interleukin IL-23/IL-17 axis. The enthesis functions as a key organ in which mechanical stress, microbial stimuli and genetic predisposition trigger local immune activation. This leads to the development of a systemic inflammatory response involving different clinical domains. Genetic and immunological heterogeneity explain the diversity of phenotypes and treatment responses and underpins the perspective of endotype-based stratification for precision medicine approaches.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149064852","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":"[Oxalate-rare crystal disease in joints and kidneys].","authors":"Franca S Deicher, Jingfang Zhou-Stache, Raoul Bergner, Jörg H W Distler, Wolfgang Merkt","doi":"10.1007/s00393-026-01872-8","DOIUrl":"https://doi.org/10.1007/s00393-026-01872-8","url":null,"abstract":"<p><p>We present the case of a 75-year-old patient who presented with recurrent arthralgia. Additional findings included chronic renal impairment of unknown cause and a history of partial small bowel resection. Arthrosonography raised suspicion of crystal arthropathy; however, no crystals were detected in the knee aspirate. Further investigation revealed previous findings of oxalate nephropathy, most likely as a result of increased reabsorption following abdominal surgery. Subsequently, Von Kossa staining of the aspirate confirmed oxalate arthropathy.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149040956","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":"The MHC-I-opathy architecture: immunogenetic and biomechanical determinants of disease expression.","authors":"Kerem Abacar, Dennis McGonagle","doi":"10.1007/s00393-026-01865-7","DOIUrl":"https://doi.org/10.1007/s00393-026-01865-7","url":null,"abstract":"<p><p>Major histocompatibility complex class I molecule (MHC-I)-opathies were originally proposed to unify clinically heterogenous diseases such as spondyloarthritis, psoriasis and Behcet's disease, in which MHC class I genetics, endoplasmic reticulum aminopeptidase (ERAP)-dependent peptide processing, barrier dysfunction, mechanical stress and CD8 T‑cell responses intersect. In this review, we argue that this concept has become increasingly defined and should now be considered as a graded pathogenic framework. Within this model, type 17 MHC-I-opathies, including psoriasis and spondyloarthritis, are largely coupled to interleukin (IL)-23/IL-17-driven neutrophilic inflammation, whereas Takayasu arteritis may represent a type 1 MHC-I-opathy in which human leucocyte antigen B*52 (HLA-B*52)-associated antigen presentation converges with IL-12B, interferon gamma (IFNγ), tumour necrosis factor (TNF) and macrophage-driven granulomatous vascular inflammation. Disease expression is shaped not by MHC‑I carriage alone, but by the extent to which multiple genetic, environmental and tissue-specific layers align the HLA/ERAP-mediated peptide repertoire, barrier perturbation, microbiota- or tissue-derived antigenic pressure, biomechanical stress and adaptive CD8 T‑cell effector programmes. The more completely these layers converge, the more likely a disease or patient is to display the characteristic behaviour of an MHC-I-opathy, including earlier onset, recurrent tissue-localised inflammation, broader anatomical involvement and greater severity. This framework moves the field beyond susceptibility odds ratios toward phenotype stratification, prognostic prediction and rational selection of targeted therapies, including cytokine blockade, modulation of antigen presentation and approaches directed at pathogenic CD8 T‑cell repertoires.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031553","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":"[Eyelid edema as presenting manifestation of systemic sarcoidosis].","authors":"Paul Jung, Thomas Schneider, Rasmus Leistner, Hildrun Haibel","doi":"10.1007/s00393-026-01870-w","DOIUrl":"https://doi.org/10.1007/s00393-026-01870-w","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031575","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":"Adult-onset Still's disease in an 83-year-old patient with positive antinuclear antibodies: a case report.","authors":"Ruibing Deng, Xin Jia, Wei Zhang, Panghua Ding, Jingqi Duan, Xiaoming Wang","doi":"10.1007/s00393-026-01841-1","DOIUrl":"https://doi.org/10.1007/s00393-026-01841-1","url":null,"abstract":"<p><p>Adult-onset Still's disease (AOSD) is a rare, systemic autoinflammatory disorder predominantly affecting young adults. It is characterized by polyarthritis, systemic inflammation with high spiking fevers, a transient salmon-pink rash, hepatosplenomegaly, and elevated ferritin levels. Its clinical presentation is highly heterogeneous and lacks specificity, and there are currently no unified diagnostic criteria. Differential diagnosis requires the exclusion of infections, malignancies, other rheumatic diseases, and adverse drug reactions. We report the case of a woman in her 80s in whom the diagnosis of adult-onset Still's disease was delayed owing to her advanced age, atypical rash, and positive antinuclear antibody status, despite significantly elevated serum ferritin levels. This case highlights that AOSD can affect older individuals, including those aged ≥ 80 years, and should be considered in the differential diagnosis of these patients when presenting with unexplained fever, especially if accompanied by rashes and hyperferritinemia. Moreover, a positive antinuclear antibody test does not rule out the diagnosis of AOSD. Timely and appropriate treatment can improve the prognosis of older patients with adult-onset Still's disease.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921012","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":"[Diagnostics of Sjögren's disease].","authors":"Stephanie Finzel","doi":"10.1007/s00393-026-01801-9","DOIUrl":"10.1007/s00393-026-01801-9","url":null,"abstract":"<p><strong>Background: </strong>Sjögren's disease (SjD) is a chronic inflammatory autoimmune disorder characterized by heterogeneous clinical manifestations and variable systemic involvement. An accurate diagnosis requires a structured, multimodal diagnostic approach.</p><p><strong>Objective: </strong>Presentation of the current diagnostic strategies in suspected SjD, focusing on the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria, objective functional diagnostics, serological markers, histopathology, imaging modalities and domain-specific diagnostics within the EULAR Sjögren's syndrome disease activity index (ESSDAI) framework.</p><p><strong>Methods: </strong>Narrative review based on current classification criteria, validated diagnostic tools, imaging techniques, key publications and consensus statements.</p><p><strong>Results: </strong>The ACR/EULAR classification criteria published in 2016 represent the current international standard for classification of SjD. Objective functional measurement of lacrimal and salivary glands, serological testing (particularly anti-SSA/Ro antibodies), labial salivary gland biopsy, and imaging modalities provide complementary diagnostic information. Salivary gland ultrasonography has emerged as a valuable noninvasive tool. The ESSDAI enables standardized assessment of systemic disease activity and requires tailored diagnostic procedures depending on the affected organ domains.</p><p><strong>Conclusion: </strong>The diagnostics of SjD rely on the integration of classification criteria and domain-specific assessments. A personalized, multimodal diagnostic strategy is essential for accurate diagnosis, disease activity evaluation and making informed therapeutic decisions.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"520-526"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147718495","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":"[Epidemiology and manifestations of Sjögren's disease].","authors":"Martin Aringer, Anne-Kathrin Tausche","doi":"10.1007/s00393-026-01796-3","DOIUrl":"10.1007/s00393-026-01796-3","url":null,"abstract":"<p><p>While the name of Sjögren's syndrome was recently changed to Sjögren's disease (SjD), the picture of a systemic autoimmune disease with inflammation of the exocrine glands and subsequent dryness has remained unchanged since the first description. This includes a pronounced B‑cell activity with an increased risk of lymphoma, frequent fatigue and arthralgia and relevant organ involvement in some patients. The prevalence in Germany is estimated to be around 7:10,000, slightly higher than for systemic lupus erythematosus (SLE). Women are affected roughly ten times more often than men. The classification criteria are based on antibodies against Ro/SS‑A antigens or a salivary gland biopsy, supplemented by the objective proof of dryness. In view of the now well-established and typical sonographic findings, they could be supplemented by sonographic parameters.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"505-510"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147366629","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}