Zeitschrift fur Rheumatologie最新文献

Over the last 40 years, the clinical picture of chronic osteomyelitis without a microbiological cause has increasingly been described as chronic nonbacterial osteomyelitis/osteitis (CNO) in the sense of an entity. With the sharpening of the clinical picture, the disease designations became increasingly homogeneous. Consequently, an internationally agreed classification of CNO for childhood has recently been published, which is based on two studies on clinical diagnostic criteria. With regard to pathophysiology, findings have also shown that CNO can be categorised as an auto-inflammatory disease. However, it remains a diagnosis of exclusion. Quite a few differential diagnostic entities must be considered, which are almost indistinguishable from genuine CNO both clinically and morphologically. For the latter, a genetic or metabolic cause is still largely unknown, although various activations of inflammatory pathways have been described. In the meantime, the basis has been laid for conducting controlled studies in which, in addition to the classification, parameters for disease activity and suggestions for inactive disease and remission have been published.This review describes the historical development towards a now more clearly defined disease. It discusses diagnostic and therapeutic algorithms using the example of an adolescent patient with spinal and extremity manifestations.

Background: The post-COVID syndrome (PCS) describes long-lasting symptoms after a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. PCS and rheumatic diseases, especially collagenoses, show strong overlap of symptoms and biomarkers. Thus far, no biomarkers that differentiate between PCS patients with and without rheumatic diseases exist, and data on the prevalence of rheumatic diseases in this collective in Germany is scarce.

Method: Based on the online platform DEFEAT-Corona, 80 people with PCS without a previously confirmed inflammatory rheumatic disease (IRD) and interest in a rheumatological evaluation were recruited. Typical complaints of PCS and rheumatic diseases were analyzed. In addition, comprehensive laboratory analyses were conducted.

Results: In 6.25% (n = 5) of the PCS patients,IRD was suspected or confirmed. The remaining 75 PCS patients without IRD also showed a high degree of overlap with regard to complaints typical for rheumatic disease or PCS. The inflammation parameters C‑reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were significantly higher in PCS patients with suspected IRD compared to patients with PCS only and significantly more often exceeded normal range.

Conclusion: This study illustrates the high degree of overlap between PCS and rheumatic symptoms in PCS patients without previous suspicion of IRD. The risk for IRD could be elevated in PCS. However, in the view of the authors, PCS without additional risk factors, such as elevated CRP or arthritis, does not generally justify rheumatological evaluation in clinical routine. This recommendation should be further investigated in larger studies.

Background: Systemic sclerosis (SSc) is a complex autoimmune disease with multiorgan involvement and substantial morbidity and mortality. As a result, patients with SSc are disproportionately affected by osteoporosis, which remains insufficiently characterized in the context of SSc and is often underrecognized in clinical practice.

Objective: To present the current state of evidence regarding the epidemiology, pathophysiological mechanisms, diagnostic features, and therapeutic challenges of osteoporosis in the context of SSc.

Methods: Narrative review of relevant primary and secondary literature (2003-2025) from PubMed, Embase, and current guideline sources, focusing on osteological aspects in SSc.

Results: Patients with SSc exhibit a significantly increased prevalence of osteoporosis and fractures. SSc aggravates osteoporosis risk factors such as postmenopausal status, malnutrition and low body weight through vasculopathy, fibrosis, as well as autoimmunity/inflammatory activity. The trabecular bone score (TBS) serves as a useful adjunct in fracture risk assessment. Furthermore, SSc complicates, especially due to gastrointestinal involvement, osteospecific therapy, with oral bisphosphonates appearing less effective. Preliminary evidence supports the effectiveness of parenteral antiresorptive agents.

Conclusion: Osteoporosis in SSc requires disease-specific risk assessment and an individualized diagnostic and therapeutic approach. Further prospective studies are needed to establish evidence-based treatment recommendations.

Transarterial periarticular embolization (TAPE) is a novel interventional procedure for local pain therapy in osteoarthritis. The reduction of local hypervascularization is achieved by supraselective embolization of periarticular arteries using embolic agents. The procedure described herein results in a reduction in both pain and the use of analgesics. The drug was originally developed for the treatment of knee osteoarthritis and initial small studies and case reports indicated that it was effective for other joints, including the fingers and shoulders. In the domain of rheumatology TAPE opens up new therapeutic perspectives, particularly for cases of treatment-resistant monoarthritis and arthritis associated with checkpoint inhibitors.

The targeted and personalized cell and gene therapy with autologous chimeric antigen receptor (CAR) transduced T‑cells (CAR T‑cells) has become established as the standard therapy for patients with B‑cell diseases, such as CD-19 positive leukemia and lymphomas and B‑cell maturation antigen (BCMA) positive multiple myeloma (MM). This therapy has been approved in Europe since 2018 and is administered in more than 50 clinics in Germany. With increasing experience and expertise in understanding of the principles of action and underlying mechanisms as well as mitigation of side effects have significantly improved. In the near future expansion of CAR T‑cell applications are to be expected, aimed at addressing further targets and therefore other disease entities. Currently, the indications have already expanded beyond hematology and oncology to rheumatology, neurology and other fields.

Background: Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking.

Objective: The aim of the study was to assess the current status of ILD-MDM in German hospitals to gain insights into their composition, processes and potential for optimization.

Material and methods: A web-based survey was conducted via SurveyMonkey under the auspices of the German Society for Rheumatology and Clinical Immunology (DGRh) and in collaboration with the German Respiratory Society (DGP) and the German Radiological Society (DRG). A standardized questionnaire captured information on the participating specialist disciplines, organizational structures as well as the content and challenges of local ILD-MDM. The analysis was conducted descriptively.

Results: A total of 125 physicians from 15 federal states in Germany participated. Pulmonologists (93.6%), radiologists (86.4%), rheumatologists (59.2%) and pathologists (57.6%) are the most commonly represented members of ILD-MDM. The majority of ILD-MDMs are conducted either in person (50%) or in a hybrid format (31.5%) and are held on a weekly basis (41.1%). Of all patient cases discussed, two thirds receive a definitive diagnosis and treatment recommendation.

Conclusion: The findings reveal a high acceptance and prevalence of ILD-MDM in Germany but also highlight potential areas for improvement, particularly regarding interdisciplinary participation, technical infrastructure and standardization.

The treatment of fibrosing autoimmune diseases has so far shown no significant progress with respect to fibrosis. The reason for this is unclear. As in vitro and in vivo data have shown that B‑lymphocytes are not only responsible for autoantibody production but also play an important role in the activation of fibroblasts in an inflammatory event, depletion of B cells is meaningful in these fibrosing autoimmune diseases. The use of the CD20 antibody rituximab has shown some therapeutic benefits but could not decisively improve fibrosis, the prognosis-relevant complication in these diseases. This could be due to the insufficient tissue access of a soluble antibody and the resulting limited depletion of B‑lymphocytes in the inflammatory area of connective tissue. This is the decisive advantage of a cell-mediated destruction of autoimmune cells, which is possible via autologous B‑cell-directed chimeric antigen receptor (CAR) T‑cell therapy. The first data on the treatment of non-SLE connective tissue diseases with fibrosing aspects with autologous CAR T‑cells are developed based on this idea. This overview summarizes and discusses these data. It is important to coordinate research aspects of these experimental treatment approaches and the most important open questions, in our opinion, are proposed at the end of the review.

Background: Chimeric antigen receptor (CAR) T cell therapies were originally developed for the treatment of hematological malignancies; however, they are gaining increasing importance in the treatment of selected individuals with severe, treatment-refractory courses of neuroimmunological diseases. This article discusses the available treatment experiences to date and the potentially promising biotechnological developments in the context of the underlying neuroimmunological pathophysiology.

Observations: The spectrum of immunopathology in neuroimmunological diseases ranges from classical autoantibody-mediated autoimmune diseases, such as myasthenia gravis to immunologically complex conditions like multiple sclerosis. The CAR T cell products currently in use target B cells, leading to complete B cell depletion, including autoreactive B cell clones. The therapeutic response, measured by disease activity and biomarkers, varies depending on the underlying immunopathology. The use of CAR T cells in different disease entities has shown a favorable safety profile concerning acute toxicity.

Conclusion: Currently available and emerging CAR T cell therapy approaches open new therapeutic perspectives for neuroimmunological diseases. Larger studies are needed to assess safety, efficacy and long-term effects and to identify individual disease courses that may be suitable for the application of these forms of treatment.