International Journal of Rheumatic Diseases最新文献

Background/purpose: Our objective was to investigate real-world outcomes and treatment strategies in individuals affected by DADA2 using over 10-year period real-life experience.

Methods: This descriptive analysis encompassed all adult/pediatric patients with DADA2 from our Vasculitis Research Center prospective database. Patients on anti-TNF therapy have been specifically examined, analyzing the treatment's duration, indications, and outcomes. Treatment responses were based on physicians' assessments and categorized as full response (symptom-free with normal acute phase reactants) or partial/no response.

Results: Totally 32 patients (Adult/Childhood age: 19/13) were analyzed. Anti-TNF agents were prescribed to 27 of the 32 patients. Over a median follow-up of 58 months on anti-TNF therapy, 10 patients (35.7%) exhibited a complete response, predominantly in cases with nervous system or skin involvement. Partial responses were observed in the other 10 patients (35.7%). Currently, 20/ 27 patients remain on anti-TNF treatment. Among the seven who are not on anti-TNF now: five died (four of them with a late diagnosis, one could not continue due to cardiomyopathy), one refused treatment and one had a cure after bone marrow transplantation. We have become aware that four patients increased their dose interval and one returned to the normal interval after an increase in CRP. The first patient was diagnosed in 2013 and over the last 10 years, 6/32 (18.8%) of the patients died.

Conclusion: Anti-TNF treatment is beneficial for vasculitic and inflammatory lesions. The clinical course of patients is diverse, especially if the diagnosis is delayed, with a mortality rate of up to 20% over a 10-year period.

Background: Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by the destruction of exocrine glands primarily via T-cell-mediated B-cell over-activation and cytokine production. This leads to pronounced dryness of the mouth and eyes and can result in multi-systemic involvement affecting the kidneys, lungs, and blood. In recent years, there has been increasing attention on the role of immune cells in pSS. However, studies investigating the causal role of immune cells in pSS have been relatively limited.

Methods: In this study, we employed a two-way two-sample Mendelian randomization approach to assess the causal relationship between immune cells and pSS. Utilizing publicly available genome-wide association study (GWAS) data, we explored the causal links between 731 immunophenotypically labeled immune cells and the risk of pSS.

Results: Through the use of instrumental variables derived from GWAS data and corrected for false discovery rate (FDR), we identified three immune cells with increased levels that were causally associated with pSS risk (FDR < 0.05). These included IgD+ CD38br AC B cells, CD27 on IgD+ CD38- unswitched memory B cells, and Granulocyte % leukocyte. Additionally, three immune cells with reduced levels were found to be causally associated with pSS risk, namely CD4+ CD8dim %lymphocyte, CD4+ CD8dim %leukocyte, and CD28 on activated and secreting regulatory T cells (Tregs). Furthermore, the development of pSS was associated with elevated levels of CD33br HLA DR+ CD14- % CD33br HLA DR+ in myeloid cells.

Conclusion: This study demonstrates that immune responses influence the progression of pSS in a complex pattern. Our findings may provide new insights into the immunology of pSS pathogenesis and more experimental studies should be conducted to further explore the potential mechanisms between identified immune features and pSS risk, which may provide a basis for exploring early intervention methods for pSS and developing targeted therapeutic strategies or even reshaping immune homeostasis.

Rheumatoid arthritis (RA) is an autoimmune condition that mostly impacts the joints. During the advanced phases of the disorder, it may be accompanied by other problems. While the precise cause of RA is uncertain, various research has been conducted to gain a better understanding of the immunological processes involved in the development of RA. T cells are acknowledged as significant contributors to the progression of RA because of their roles in cytokine secretion, antigen presentation, and facilitating B cells in the manufacture of antibodies. γδ T cells are a small subset of T cells that have significant functions in the context of infection and diseases linked with tumors. γδ T cells have been the subject of investigation in autoimmune disorders in recent years. This review focused on the involvement of γδ T lymphocytes in the development of RA. In this article, we provide an analysis of the immunological capabilities of γδ T cells, intending to comprehend their significance in RA, which could be pivotal in the creation of innovative clinical treatments.

Exercise has numerous health benefits in patients with autoimmune inflammatory rheumatic diseases (AIRDs). Regular physical activity can help maintain/improve bone health. The aim of the present article was to review current knowledge on the effects of exercise on bone health in patients with AIRDs, particularly in those experiencing a high corticosteroid burden. The article also aimed to discuss potential mechanisms underlying the benefits of physical activity/exercise on bone tissue. Potential explanations regarding the role of exercise on bone health in AIRDs include anti-inflammatory effects, mechanical loading, improvement in muscle strength, hormonal changes, improvement in balance, and effects on telomere erosion, deoxyribonucleic acid methylation, and gene expression. Current evidence regarding the outcomes of exercise on bone health in patients with AIRDs is predominantly derived from studies focused on rheumatoid arthritis. Expanding research to include other rheumatic conditions would enhance the overall understanding of this topic.