Zeitschrift fur Rheumatologie最新文献

Background: Systemic rheumatic diseases with interstitial lung disease (SARD-ILD) are often associated with progressive pulmonary fibrosis (PPF), characterised by increasing respiratory symptoms, progressive loss of lung function, and an unfavourable prognosis. Currently available therapies, including glucocorticoids, immunosuppressants and antifibrotic agents, often have limited efficacy depending on the underlying SARD-ILD, while adverse events limit their use. Therefore, there is a substantial unmet need for effective and well-tolerated treatment options.

Objectives: This review summarises current developments and emerging therapeutic approaches in SARD-ILD and PPF, highlighting their potential relevance for clinical practice.

Materials and methods: A systematic PubMed search (publications up to mid-2025) using the terms "SARD-ILD", "CTD-ILD", "PPF" and "treatment" identified relevant studies investigating novel therapeutic strategies.

Results: Substances currently under clinical investigation include nerandomilast, belimumab, admilparant, anifrolumab and inhaled treprostinil. Nerandomilast, a preferential PDE4B inhibitor with antifibrotic and anti-inflammatory properties, demonstrated a reduction in lung function loss and mortality risk with good tolerability in the phase‑3 FIBRONEER-ILD study, potentially representing a turning point in the management of autoimmune-associated ILD.

Conclusion: Promising results from ongoing clinical studies provide new therapeutic perspectives for SARD-ILD and PPF and may significantly improve prognosis and disease burden in the future.

Medical knowledge is growing exponentially in rheumatology, posing increasing challenges for knowledge dissemination among physicians, educators and patients. Traditional information and learning formats are reaching their limits in view of the rapid emergence of new clinical studies, guidelines and treatment concepts. Large language models (LLMs) offer the possibility to structure, synthesize and contextually adapt extensive and complex information within a short time. This opens new perspectives for clinical decision support, medical education, patient education and scientific work in rheumatology. This article systematically categorizes the use of LLMs across these fields of application and discusses the opportunities, risks and practical implications using selected examples that colleagues can immediately apply. Early data suggest a high potential for use and growing acceptance among physicians, students and patients; however, significant challenges remain. These include concerns regarding the validity and transparency of the generated content, potential biases, data protection issues and the risk of uncritical adoption of artificial intelligence (AI)-generated recommendations. The use of LLMs can make rheumatological knowledge rapidly, individually, and easily accessible but does not replace medical responsibility or clinical expertise. An evidence-based evaluation, clear regulatory framework conditions, safe integration into existing workflows and targeted training and governance concepts are essential to harness the potential of LLMs for knowledge dissemination in rheumatology in a sustainable and responsible manner.

Ultrasound in medicine has a history arbitrarily going back to the days when a modified custom-built metal flaw detector was used in post-War Glasgow to diagnose abdominal masses. Spanning a period of 30 years, the article describes the coming of age of ultrasound within the field of rheumatology. This historical paper highlights collaborative research focusing on validation and standardization of the imaging modality. In addition, the work of several persons who have played a key role in the development of ultrasound in the field of rheumatology is emphasized. In real time and at the point of care, ultrasound can demonstrate where inflammation is present, thereby giving doctors and patients a better idea of why and where pain and stiffness originate. In retrospect, we may conclude that ultrasound has profoundly changed the practice of clinical rheumatology.

Background: Sjögren's syndrome is now referred to internationally as Sjögren's disease (abbreviated SjD).

Objectives: The aim was to reach a consensus on how Sjögren's syndrome should be referred to in German-speaking countries in the future.

Material and methods: The authors of this manuscript were appointed by the three professional associations in the D‑A-CH region, the German Society for Rheumatology and Immunology (DGRh), the Austrian Society for Rheumatology (ÖGR) and the Swiss Society for Rheumatology (SGR) to implement the international recommendations for the German-speaking region.

Results: The term "Sjögren-Erkrankung" should replace "Sjögren-Syndrom." The English abbreviation "SjD" (Sjögren's disease) should also be used in German as an abbreviation for "Sjögren-Erkrankung." The term "associated" should be used instead of "secondary" for Sjögren's disease that occurs in conjunction with another classifiable systemic autoimmune disease.

Background: Primary Sjögren's disease (SjD) is a chronic autoimmune disease that predominantly affects exocrine glands but can also show numerous systemic organ manifestations.

Objective: 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.

Methods: Selective literature review of current original articles, reviews and clinical studies.

Results: 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.

Discussion: 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.

Alongside the editorial control mechanisms of traditional media, such as fact-checking and source verification, medical journalism ensures the quality of medical knowledge transfer, similar to the peer-review process of professional journals. However, this role, which is central to a democracy, is increasingly threatened by the rise of the internet and social media. Today, most news from science and medicine reaches citizens as "end consumers" of information directly, i.e., without intermediary quality control according to established journalistic standards. Deteriorating working conditions have forced many medical journalists to move to other occupational fields. The loss of quality control in medical knowledge transfer has become a danger to society. It has never been so difficult to distinguish between reliable information and misinformation or disinformation.