Michael Gernert, Eva Christina Schwaneck, Marc Schmalzing
{"title":"[T-cell large granular lymphocytic leukemia and Felty's syndrome in rheumatoid arthritis].","authors":"Michael Gernert, Eva Christina Schwaneck, Marc Schmalzing","doi":"10.1007/s00393-024-01611-x","DOIUrl":"https://doi.org/10.1007/s00393-024-01611-x","url":null,"abstract":"<p><p>Neutropenia in rheumatoid arthritis (RA) is a problem that often needs to be addressed. Side effects of basic antirheumatic treatment, infections or substrate deficiencies are common causes; however, T‑cell large granular lymphocytic (T-LGL) leukemia, a mature T‑cell neoplasm, can also lead to autoimmune cytopenia. The T‑LGL leukemia can be associated not only with RA but also with other autoimmune diseases or neoplasms. Correspondingly, increases in clonal T cells, natural killer T (NKT) cells and LGL cells are found in the peripheral blood. A T‑cell receptor PCR and flow cytometry (or at least a blood smear) are therefore necessary to diagnose T‑LGL leukemia. The presence of clonal T cells alone is usually not pathological. A distinction must be made from Felty's syndrome (consisting of the clinical triad of arthritis, leukopenia, splenomegaly), which does not require the two T‑LGL leukemia criteria mentioned. The treatment for both entities (with underlying RA) is methotrexate and, if insufficiently effective, rituximab.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143034573","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}
Ulla Stumpf, Ralf Schmidmaier, Hanna Taipaleenmäki, Wolfgang Böcker, Andreas Kurth, Eric Hesse
{"title":"[Influencing fracture healing by specific osteoporosis medications].","authors":"Ulla Stumpf, Ralf Schmidmaier, Hanna Taipaleenmäki, Wolfgang Böcker, Andreas Kurth, Eric Hesse","doi":"10.1007/s00393-024-01610-y","DOIUrl":"https://doi.org/10.1007/s00393-024-01610-y","url":null,"abstract":"<p><strong>Background: </strong>Osteoporosis is a widespread disease defined by a reduction in bone mass and structure, thereby increasing the risk of fragility fractures. Treatment typically involves specific medications, which either inhibit bone resorption (antiresorptive) or stimulate bone formation (anabolic) and may potentially influence the healing of osteoporotic fractures. On the other hand, metabolic disorders, immune system dysfunctions or circulatory problems can impair fracture healing. Therefore, the targeted use of osteoporosis medications could be a strategy to promote the healing of impaired fractures.</p><p><strong>Objective: </strong>The aim of this study is to provide a current overview of the effects of osteoporosis medications approved in Germany on fracture healing. The focus is on the potential influence of these medications in the context of osteoporosis treatment. Additionally, the current state of research is examined to explore to what extent the targeted use of these medications could improve fracture healing.</p><p><strong>Material and methods: </strong>A literature search was conducted in the PubMed database using topic-specific keywords. Preclinical studies, clinical trials, review articles and meta-analyses were considered to present the current scientific knowledge with clinical relevance.</p><p><strong>Results: </strong>Preclinical and clinical studies suggest that specific osteoporosis medications do not have a clinically relevant negative impact on the healing of fragility fractures. Osteoanabolic substances even tend to have a positive effect on fracture healing in both normal and impaired healing processes; however, the available studies are limited and none of the medications have been approved for this specific use.</p><p><strong>Discussion: </strong>Osteoporosis medications with antiresorptive or osteoanabolic effects are primarily used to treat osteoporosis, especially after fragility fractures, to reduce the risk of further fractures. There is no clinically relevant impairment of fracture healing due to these medications. Further studies would be required to obtain approval for these medications specifically to improve fracture healing.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980110","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":"[Obituary of the German Society for Rheumatology and Clinical Immunology for Prof. Dr. Erika Gromnica-Ihle : *06 February 1940, †15 November 2024].","authors":"Andreas Krause, Eva Seipelt","doi":"10.1007/s00393-025-01617-z","DOIUrl":"https://doi.org/10.1007/s00393-025-01617-z","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980112","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":"Global prevalence and solutions for burnout among rheumatologists.","authors":"Yoshiyasu Takefuji","doi":"10.1007/s00393-024-01613-9","DOIUrl":"https://doi.org/10.1007/s00393-024-01613-9","url":null,"abstract":"<p><p>Burnout among rheumatologists is globally prevalent, driven by low personal accomplishment, younger age, dissatisfaction with the specialty, low income, long hours, emotional exhaustion, and depersonalization. Mitigation strategies include addressing modifiable risk factors, implementing organizational measures, investing in well-being, assessing individual grit, and managing workload with virtual care platforms.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980135","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":"[Quintessence of the new guidelines on physical training and fracture prophylaxis].","authors":"Uwe Lange, Anett Reißhauer, Wolfgang Kemmler","doi":"10.1007/s00393-024-01609-5","DOIUrl":"https://doi.org/10.1007/s00393-024-01609-5","url":null,"abstract":"<p><p>The guidelines on physiotherapy and exercise for osteoporosis from 2008 have recently been extensively revised on the basis of new scientific findings and possible applications. The S3 guidelines have not yet been approved by consensus and the planned completion date (Association of the Scientific Medical Societies in Germany, AWMF online, registry number 183-002) is autumn 2024. Based on the publication in Issue 3 Osteology (August 2023), key points of the guidelines on physical training and fracture prophylaxis are summarized in abridged form. These are practice-oriented, evidence-based recommendations for optimal training for fracture prevention.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980126","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":"[Septic musculoskeletal complications under immunomodulating treatment].","authors":"Anna Kernder, Christian Kneitz","doi":"10.1007/s00393-024-01595-8","DOIUrl":"https://doi.org/10.1007/s00393-024-01595-8","url":null,"abstract":"<p><p>Infections are an important cause of morbidity and mortality in patients with inflammatory rheumatic diseases. Among these, musculoskeletal infections represent a relevant proportion as patients with rheumatoid arthritis face an increased risk of developing septic arthritis and prosthesis infections. The causes are multifactorial. In addition to immunosuppressive treatment, risk factors of infection in rheumatoid arthritis (RA) patients include repeated intra-articular joint punctures, an increased rate of joint replacement surgery, damaged joint structure and comorbidities. The use of glucocorticoids and tumor necrosis factor alpha (TNF-alpha) inhibitors, especially in the first 6 months of treatment, increase the risk of septic arthritis and periprosthetic joint infections. In addition, an increased disease activity could also be identified as a risk factor. Under immunosuppressive therapy rare pathogens such as Candida and mycobacteria can cause the infection and should be considered when there is a lack of clinical response to antibiotic treatment.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932818","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":"[Switching Biologics: this is how I proceed].","authors":"Peer Aries","doi":"10.1007/s00393-024-01608-6","DOIUrl":"https://doi.org/10.1007/s00393-024-01608-6","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142847774","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}
Rhea Friedrich, Anna Kernder, Norbert Blank, Diana Ernst, Jörg Henes, Gernot Keyßer, Philipp Klemm, Martin Krusche, Anna Meinecke, Jürgen Rech, Nils Schulz, Dirk Schomburg, Stefan Vordenbäumen, Eugen Feist
{"title":"Implementation of the new DGRh S2e guideline on diagnostics and treatment of adult-onset Still's disease in Germany : Implications for clinical practice in rheumatology.","authors":"Rhea Friedrich, Anna Kernder, Norbert Blank, Diana Ernst, Jörg Henes, Gernot Keyßer, Philipp Klemm, Martin Krusche, Anna Meinecke, Jürgen Rech, Nils Schulz, Dirk Schomburg, Stefan Vordenbäumen, Eugen Feist","doi":"10.1007/s00393-024-01607-7","DOIUrl":"https://doi.org/10.1007/s00393-024-01607-7","url":null,"abstract":"<p><strong>Background: </strong>Adult-onset Still's disease (AOSD) is a rare autoinflammatory disease. Since it can lead to variable organ involvement, including life-threatening complications, and due to newly available therapeutic approaches, the German Society for Rheumatology and Clinical Immunology (Deutsche Gesellschaft für Rheumatologie und klinische Immunologie; DGRh) issued a newly developed S2e guideline in December 2022.</p><p><strong>Objective: </strong>This study aims to investigate the influence of the new guideline on the diagnosis, management, and outcomes of AOSD.</p><p><strong>Methods: </strong>Retrospective data from 168 patients diagnosed with AOSD between 2007 and 2023 (92 women and 76 men; average age 40.39 years) were captured at nine centers in Germany. Patient characteristics; results of laboratory, physical, and instrumental examinations; and therapeutic regimens were analyzed at three different timepoints.</p><p><strong>Results: </strong>After publication of the German AOSD guideline, the time to diagnosis was shorter (mean before: 18.56 months, mean after: 1.29 months) and fewer complications were recorded, especially with respect to macrophage activation syndrome. Although therapeutic approaches did not change over time, treatment side effects were lower in the recent observation periods. Of note, more patients have been diagnosed with cardiac (19% to 23.1%) and pulmonary (13.8% to 23.1%) manifestations of AOSD in recent years.</p><p><strong>Conclusion: </strong>The new AOSD guideline has contributed to increased disease awareness, with earlier diagnosis and identification of extra-articular organ manifestations. Treatment side effects were less frequent, especially those related to glucocorticoids. However, there is still a need to further improve the management of AOSD.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142819508","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}
Alexander Pfeil, Martin Fleck, Martin Aringer, Xenofon Baraliakos, Diana Ernst, Isabell Haase, Christiana Hillebrecht, Bimba Franziska Hoyer, Gernot Keyßer, Ina Kötter, Andreas Krause, Martin Krusche, Hanns-Martin Lorenz, Fabian Proft, Florian Schuch, Diana Vossen, Anna Voormann, Ulf Wagner, Jürgen Wollenhaupt, Christof Specker
{"title":"[Criteria for the authorization of training in medical specialist competence in internal medicine and rheumatology-A position paper of the German Society for Rheumatology and Clinical Immunology].","authors":"Alexander Pfeil, Martin Fleck, Martin Aringer, Xenofon Baraliakos, Diana Ernst, Isabell Haase, Christiana Hillebrecht, Bimba Franziska Hoyer, Gernot Keyßer, Ina Kötter, Andreas Krause, Martin Krusche, Hanns-Martin Lorenz, Fabian Proft, Florian Schuch, Diana Vossen, Anna Voormann, Ulf Wagner, Jürgen Wollenhaupt, Christof Specker","doi":"10.1007/s00393-024-01598-5","DOIUrl":"https://doi.org/10.1007/s00393-024-01598-5","url":null,"abstract":"<p><p>The model advanced training regulations define the content of advanced training to achieve the qualification of medical specialist in internal medicine and rheumatology. There are currently no criteria for issuing the authorization in advanced training. This position paper describes the criteria proposed by the German Society for Rheumatology and Clinical Immunology (DGRh), which should be the foundation for the issuance of authorization for advanced training in the field of internal medicine and rheumatology and for the assessment of the duration. The model advanced training regulations 2018 and the advanced training plan recommended by experts function as the basis for this. Based on the criteria, the authorization for advanced training to advanced specialist training in internal medicine and rheumatology can be allocated in a standardized, graded and transparent manner throughout Germany. This enables an optimal quality of advanced training in rheumatology, which can be adapted to the future developments in the discipline.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142814382","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}
Kirsten de Groot, Elena Csernok, Diane van der Woude
{"title":"History of antineutrophil cytoplasmic autoantibodies : Milestones in rheumatology.","authors":"Kirsten de Groot, Elena Csernok, Diane van der Woude","doi":"10.1007/s00393-024-01599-4","DOIUrl":"https://doi.org/10.1007/s00393-024-01599-4","url":null,"abstract":"<p><p>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are autoimmune inflammatory small-vessel disorders with potentially life-threatening organ manifestations. Recent disease definitions and classification criteria allow distinction between granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and non-granulomatous microscopic polyangiitis (MPA). The discovery of ANCA-autoantibodies directed against proteolytic enzymes of neutrophil granules-has enabled earlier diagnosis of AAV and paved the way to stage-adapted treatments. ANCA testing initially relied on different immunofluorescence patterns, i.e., cytoplasmic ANCA (C-ANCA) vs. perinuclear ANCA (P-ANCA), in ethanol-fixed neutrophils. This is nowadays outperformed by well-standardized immunoassays against the ANCA target antigens proteinase 3 (PR3) and myeloperoxidase (MPO) for the diagnosis of small-vessel vasculitides. The discovery of ANCA has contributed substantially to unravelling the pathogenesis of AAV, which comprises neutrophil degranulation, NETosis with concurrently amplified ANCA antigen presentation, and intra- and transmural vascular inflammation involving the alternative complement system in susceptible individuals. There is a genetic disposition concerning certain HLA alleles and polymorphisms of the proteinase 3 gene. Furthermore, epigenetic modifications impact on disease activity and relapse. During follow-up, the ANCA titer is not a reliable mirror of disease activity; however, PR3-ANCA positivity is associated with a greater likelihood of relapse and a better treatment response to rituximab as compared to cyclophosphamide/azathioprine. Within the past 60 years, the discovery of ANCA has revolutionized the ability to diagnose, understand, classify, and treat AAV in a targeted manner.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142807983","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}