Zeitschrift fur Rheumatologie最新文献

{"title":"[Current treatment, foreseeable newcomer and new treatment approaches for Sjögren's disease].","authors":"Diana Ernst, Nadine Zehrfeld","doi":"10.1007/s00393-026-01815-3","DOIUrl":"10.1007/s00393-026-01815-3","url":null,"abstract":"<p><p>Although Sjögren's disease (SjD) is the most frequent connective tissue disease, there are still no approved medications for systemic treatment. Many disease-modifying antirheumatic drugs (DMARD) and biologicals have not shown sufficient efficacy in studies. The reasons for this are the complex immunological mechanisms and a high degree of clinical and immunological heterogeneity of the patients; however, significant progress has been made in recent years: for the first time 2 phase 3 studies (ianalumab and telitacicept) reported positive results. The current treatment recommendations are based on the European Alliance of Associations for Rheumatism (EULAR) guidelines (2020) and the recommendations of the British Society for Rheumatology (2024). The treatment is currently primarily based on the leading symptoms and includes consistent treatment of dry eye and dry mouth symptoms (eye and mouth care, pilocarpine if necessary). Extraglandular manifestations continue to be treated predominantly off-label with conventional DMARDs; in severe courses rituximab is used and less frequently cyclophosphamide or intravenous immunoglobulins (IVIG). New treatment approaches show consistent effects on reduction of disease activity (EULAR Sjogren's syndrome disease activity index, ESSDAI) for the first time: In particular, B‑cell-targeted therapies (ianalumab, telitacicept), FcRn inhibitors (nipocalimab, efgartigimod), CD40/CD40L blockade, kinase inhibitors and other immunomodulatory strategies have achieved significant improvements in ESSDAI and, in some cases EULAR Sjögren's syndrome patient reported index (ESSPRI) with an acceptable safety profile. Although basic measures remain indispensable, new forms of targeted treatment mark a turning point in the treatment of SjD. In particular, B‑cell and FcRn-targeted approaches open up for the first time the prospect of evidence-based systemic treatment that goes beyond mere symptom control.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"536-544"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13558335/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147783136","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":"[Practical management of Sjögren's disease in the era of new treatment options : Current value of available outcome parameters].","authors":"Jürgen Braun, T Dörner","doi":"10.1007/s00393-026-01799-0","DOIUrl":"10.1007/s00393-026-01799-0","url":null,"abstract":"<p><p>Sjögren's disease is a chronic inflammatory autoimmune disease, which mostly affects women who suffer particularly from dryness of mucous membranes, especially in the eyes and the mouth, tiredness and potentially also extraglandular manifestations such as joint and muscle pain. In the initial diagnostic work up serological alterations especially autoantibodies against SS-A(Ro)/SS-B(La) and rheumatoid factors play an important role. The pathogenesis is not yet finally clarified but an important role of B cells can be assumed. To date mostly symptomatic treatment options to improve glandular functional deficits with respect to the sicca symptoms are available. Based on most recent publications there is justifiable hope that this could soon change, especially with respect to extraglandular manifestations. For this reason, it is particularly important that validated outcome parameters are available to enable proper evaluation of new treatment options. In this article the EULAR instruments Sjögren's syndrome disease activity index (ESSDAI) for documenting extraglandular manifestations and Sjögren's syndrome patient reported index (ESSPRI) as patient-reported outcome (PRO) have been translated and are here presented in detail. Both also represent the cornerstones of possible outcome instruments (STAR or CRESS) of the second generation, which are currently undergoing validation.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"527-535"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13558322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147646853","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":"[Pathophysiological aspects of primary Sjögren's disease : From epithelial activation to systemic autoimmunity].","authors":"Jacob Ritter, Thomas Dörner","doi":"10.1007/s00393-026-01797-2","DOIUrl":"10.1007/s00393-026-01797-2","url":null,"abstract":"<p><strong>Background: </strong>Primary Sjögren's disease (SjD) is a chronic autoimmune disease that predominantly affects exocrine glands but can also show numerous systemic organ manifestations.</p><p><strong>Objective: </strong>Presentation of the current understanding of the pathogenesis of the disease, focusing on genetic predisposition, epithelial activation, interferon signature and the resulting B‑T cell interaction, which form the basis for innovative treatment approaches.</p><p><strong>Methods: </strong>Selective literature review of current original articles, reviews and clinical studies.</p><p><strong>Results: </strong>Current research postulates that SjD triggers epithelial dysregulation through a combination of genetic and epigenetic predispositions, hormonal influences and possible viral triggers. This leads to the release of dsDNA, dsRNA or ssRNA, which in turn activate the innate immune system. Salivary gland epithelial cells (SGECs), plasmacytoid dendritic cells (pDCs), and monocytes produce proinflammatory cytokines and additional immune cells are recruited. The pDCs produce massive amounts of type I interferon. This results in the formation of an inflammatory microenvironment, which causes SGECs to undergo apoptosis, the release of further antigens and the recruitment of T cells. In this context, myeloid cells and SGECs produce large amounts of the cytokine B‑cell activating factor (BAFF). This promotes the further recruitment of B cells. Through Th1 cells and Tfh cells a T-B cell interaction is formed, leading to the development of ectopic germinal centers, including the induction of autoreactive B cells.</p><p><strong>Discussion: </strong>The pathophysiology of SjD is a multistage process in which the early activation of the salivary epithelium and subsequent activation of the innate immune system with IFN play a crucial role in its initiation. This is followed by activation of the adaptive immune system with a focus on T‑B cell interaction and pathological B cell activation. The chronic inflammation in SjD can be understood as positive feedback from the activation of the innate and adaptive immune systems, which innovative therapeutic approaches aim to interrupt. These comprise TLR and IFN blockade, inhibition of T‑B cell interaction via CD154/CD40 blockade (e.g., dazodalibep, iscalimab) or B cell depletion strategies including. anti-BAFF‑R and anti-CD19 CAR-T cells.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"511-519"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147481970","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":"[Sjögren's disease : Manifestations in peripheral nerves].","authors":"Franz Felix Konen, Torsten Witte, Thomas Skripuletz","doi":"10.1007/s00393-026-01805-5","DOIUrl":"10.1007/s00393-026-01805-5","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"551-554"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147487498","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":"[Sjögren's syndrome with interstitial lung disease].","authors":"Theresa Graalmann, Torsten Witte, Benjamin Seeliger","doi":"10.1007/s00393-026-01814-4","DOIUrl":"10.1007/s00393-026-01814-4","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"545-550"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147677253","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":"[Erratum to: Acute diagnostics-focus on septic arthritis].","authors":"Lukas Bönnen, Christian Heiß, Markus Rupp, Christoph Biehl","doi":"10.1007/s00393-026-01861-x","DOIUrl":"https://doi.org/10.1007/s00393-026-01861-x","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148831957","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":"Erratum to: Combination therapy with mycophenolate mofetil, nintedanib, and tocilizumab in a patient with progressive systemic sclerosis-associated interstitial lung disease.","authors":"Liangyu Chen, Xiaoxuan Li, Donghui Huang, Peisheng Chen, Pan Xu, Huihui Chen, Xiao Zhang","doi":"10.1007/s00393-026-01863-9","DOIUrl":"https://doi.org/10.1007/s00393-026-01863-9","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148832206","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":"RA-Therapie mit Januskinase-Inhibitoren – Muss wirklich über ein um 40 % erhöhtes Krebsrisiko aufgeklärt werden?","authors":"Markus Gaubitz","doi":"10.1007/s00393-026-01822-4","DOIUrl":"10.1007/s00393-026-01822-4","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"490-492"},"PeriodicalIF":1.2,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148043917","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":"Erratum to: How ultrasound changed the practice of rheumatology: echoes of a 30-year collaboration.","authors":"George A Bruyn, Richard J Wakefield, Marina Backhaus, Peter V Balint, Mario Chavez-Lopez, Emilio Filippucci, Sandrine Jousse-Joulin, Gurjit S Kaeley, David Kane, Eugene Kissin, Giorgio Lorenzon, Silvia Magni-Manzoni, Ingrid Moller, Esperanza Naredo, Carlos Pineda, Wolfgang A Schmidt, Alen Zabotti, Nanno Swen","doi":"10.1007/s00393-026-01831-3","DOIUrl":"10.1007/s00393-026-01831-3","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"487"},"PeriodicalIF":1.2,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148151717","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":"[From CAR-T to T-cell engagers : New T-cell-based treatment in rheumatology].","authors":"Matthias Pierer, David Simon","doi":"10.1007/s00393-026-01846-w","DOIUrl":"10.1007/s00393-026-01846-w","url":null,"abstract":"<p><p>Cellular forms of treatment mark a paradigm shift in the treatment of autoimmune diseases. While conventional and targeted antirheumatic treatment controls disease activity mostly by continued immunomodulation, innovative cellular approaches, especially chimeric antigen receptor (CAR) T‑cells targeting CD19, target a deep elimination of disease-relevant immune cell compartments with subsequent immunological reconstitution. This concept of an immune reset is particularly attractive for those diseases in which autoreactive B‑cells, plasmablasts, autoantibodies and misdirected lymphocyte networks are pathogenetically central. In addition, T‑cell engagers or bispecific T‑cell engagers (TCE) are now becoming of interest, which as off the shelf strategies without cellular production also enable a deep B‑cell or plasma cell depletion via T cell-mediated cytotoxicity. The most impressive clinical data so far are available for systemic lupus erythematosus (SLE), likewise accumulating indications for the efficacy in systemic sclerosis (SSc) and idiopathic inflammatory myositis (IIM). The concept is highly plausible for rheumatoid arthritis (RA) and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) but is clinically still in an early developmental stage. Initial clinical experiences are available for TCE, especially for refractory RA and for severe connective tissue diseases, including SSc and antisynthetase syndrome. The evidence for other rheumatological indications is so far much more limited. This article discusses the biological principles, production and mechanisms of action, the revolutionary potential of these strategies, current evidence in selected rheumatological indications and open questions on safety, patient selection and future perspectives.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"430-439"},"PeriodicalIF":1.2,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148621619","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}