Spanish cohort of VEXAS syndrome: clinical manifestations, outcome of treatments and novel evidences about UBA1 mosaicism
Conclusion
Thirty patients with adult-onset AID were definitively diagnosed with VEXAS syndrome through genetic analyses. Despite minor interindividual differences, their main characteristics were in concordance with previous reports. We detected for the first time the UBA1 mosaicism in non-haematopoietic tissue, which questions the previous concept of myeloid-restricted mosaicism and may have conceptual consequences for the disease mechanisms. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Mascaro, J. M., Rodriguez-Pinto, I., Poza, G., Mensa-Vilaro, A., Fernandez-Martin, J., Caminal-Montero, L., Espinosa, G., Hernandez-Rodriguez, J., Diaz, M., Rita-Marques, J., Sanmarti, R., Castaneda, S., Colunga, D., Coto-Hernandez, R., Fanlo, P., Elejald Tags: Open access, ARD, Autoinflammatory disorders Source Type: research
Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study
Conclusion
These results suggest that dupilumab may be effective in treating patients with EGPA-related ENT manifestations. However, EGPA flares occurred in one-third of patients and were preceded by eosinophilia in 88%, suggesting that caution is required. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Molina, B., Padoan, R., Urban, M. L., Novikov, P., Caminati, M., Taille, C., Neel, A., Bouillet, L., Fraticelli, P., Schleinitz, N., Christides, C., Moi, L., Godeau, B., Knight, A., Schroeder, J. W., Marchand-Adam, S., Gil, H., Cottin, V., Durel, C.-A., G Tags: ARD Lay summaries, ARD, Vasculitis Source Type: research
Benralizumab for eosinophilic granulomatosis with polyangiitis
Conclusions
Benralizumab appears to be an effective treatment for refractory asthma or ENT manifestations in EGPA and allows GC-sparing. However, its efficacy was lower after prior failure of mepolizumab. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Cottu, A., Groh, M., Desaintjean, C., Marchand-Adam, S., Guillevin, L., Puechal, X., Beaumesnil, S., Lazaro, E., Samson, M., Taille, C., Durel, C.-A., Diot, E., Nicolas, S., Guilleminault, L., Ebbo, M., Cathebras, P., Dupin, C., Yildiz, H., Belfeki, N., P Tags: ARD, Vasculitis Source Type: research
Single-cell analysis reveals key differences between early-stage and late-stage systemic sclerosis skin across autoantibody subgroups
Conclusions
We have identified cellular differences between the two main autoantibody subsets in dcSSc (ARA+ and ATA+). These differences reinforce the importance of considering autoantibody and stage of disease in management and trial design in SSc. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Clark, K. E. N., Xu, S., Attah, M., Ong, V. H., Buckley, C. D., Denton, C. P. Tags: Open access, ARD, Systemic sclerosis Source Type: research
HLA-B27, axial spondyloarthritis and survival
Discussion
AS patients, but not nr-axSpA patients, have a significantly shortened life expectancy. Increased mortality is particularly significant among women with HLA-B27-positive AS. HLA-B27 carriage in the European-ancestry general population does not influence survival, or the risk of death due to vascular disease. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Li, Z., Khan, M. K., van der Linden, S. M., Winkens, B., Villiger, P. M., Baumberger, H., van Zandwijk, H., Khan, M. A., Brown, M. A. Tags: Editor's choice, ARD, Spondyloarthritis Source Type: research
Directed differentiation of human pluripotent stem cells into articular cartilage reveals effects caused by absence of WISP3, the gene responsible for progressive pseudorheumatoid arthropathy of childhood
Conclusions
WISP3-deficient and WISP3-sufficient hPSCs can be differentiated into articular cartilage-like tissues, but these tissues differ in their transcriptomes and in the relative abundances of chondrocyte subtypes they contain. These findings provide important starting points for in vivo studies when an animal model of PPAC or presymptomatic patient-derived articular cartilage becomes available. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Li, C., Alemany-Ribes, M., Raftery, R. M., Nwoko, U., Warman, M. L., Craft, A. M. Tags: ARD, Early arthritis Source Type: research
Disease activity drives transcriptomic heterogeneity in early untreated rheumatoid synovitis
Conclusion
In this large series of early, untreated RA, we show that the synovial transcriptome closely mirrors clinical disease activity and correlates with synovial inflammation. Intriguingly, lower inflammation and disease activity are associated with higher ratios of M2:M1 macrophages, particularly striking in the synovial lining layer. This may point to a protective role for tissue resident macrophages in RA. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Triaille, C., Tilman, G., Sokolova, T., Loriot, A., Marchandise, J., De Montjoye, S., Nzeusseu-Toukap, A., Meric de Bellefon, L., Bouzin, C., Galant, C., Durez, P., Lauwerys, B. R., Limaye, N. Tags: Open access, ARD, Early arthritis Source Type: research
Anti-GM-CSF otilimab versus sarilumab or placebo in patients with rheumatoid arthritis and inadequate response to targeted therapies: a phase III randomised trial (contRAst 3)
Conclusions
Otilimab demonstrated an acceptable safety profile but failed to achieve the primary end point of ACR20 and improve secondary end points versus placebo or demonstrate non-inferiority to sarilumab in this patient population.
Trial registration number
NCT04134728. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Taylor, P. C., Weinblatt, M. E., McInnes, I. B., Atsumi, T., Strand, V., Takeuchi, T., Bracher, M., Brooks, D., Davies, J., Goode, C., Gupta, A., Mukherjee, S., OShea, C., Saurigny, D., Schifano, L. A., Shelton, C., Smith, J. E., Wang, M., Wang, R., Watts Tags: Open access, ARD, Rheumatoid arthritis Source Type: research
Anti-GM-CSF otilimab versus tofacitinib or placebo in patients with active rheumatoid arthritis and an inadequate response to conventional or biologic DMARDs: two phase 3 randomised trials (contRAst 1 and contRAst 2)
Conclusions
Although otilimab demonstrated superiority to placebo in ACR20, CDAI LDA and HAQ-DI, improved symptoms, and had an acceptable safety profile, it was inferior to tofacitinib.
Trial registration numbers
NCT03980483, NCT03970837. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Fleischmann, R. M., van der Heijde, D., Strand, V., Atsumi, T., McInnes, I. B., Takeuchi, T., Taylor, P. C., Bracher, M., Brooks, D., Davies, J., Goode, C., Gupta, A., Mukherjee, S., OShea, C., Saurigny, D., Schifano, L. A., Shelton, C., Smith, J. E., Wan Tags: Open access, ARD, Rheumatoid arthritis Source Type: research
Contributions of joint damage-related events to gout pathogenesis: new insights from laboratory research
Epidemiological and imaging findings indicate that gout frequently affects damaged joints. Recent studies suggest that the relationship between gout and joint damage may be more complex than a simple unidirectional link and that joint damage may promote the development of gout at affected sites. In this article, we review the clinical associations and recent laboratory research identifying events in the setting of osteoarthritis or joint injury that can alter the intraarticular microenvironment and locally regulate monosodium urate crystallisation and deposition or amplify the inflammatory response to deposited crystals. T...
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Xu, H., Qin, H., Hua, Y., Dalbeth, N. Tags: ARD Reviews Source Type: research
Potential for biosimilars in rheumatology in Africa
Biosimilars are products which are highly similar to a reference biologic product (RBP). In Africa, regulatory frameworks for biosimilar approval are still in development in many countries and few biosimilars for rheumatic diseases are currently available. The use of biosimilar medicines in Africa provides an important opportunity to treat more rheumatology patients with biologic drugs. This editorial aims to shed a light on the potential benefits, challenges and current efforts, regarding the use of biosimilars in Africa in Rheumatology. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Abu-Zaid, M. H., Adebajo, A., El Miedany, Y. Tags: ARD Viewpoint Source Type: research
Time to treat the climate and nature crisis as one indivisible global health emergency
Over 200 health journals call on the United Nations (UN), political leaders and health professionals to recognise that climate change and biodiversity loss are one indivisible crisis and must be tackled together to preserve health and avoid catastrophe. This overall environmental crisis is now so severe as to be a global health emergency. The world is currently responding to the climate crisis and the nature crisis as if they were separate challenges. This is a dangerous mistake. The 28th Conference of the Parties (COP) on climate change is about to be held in Dubai while the 16th COP on biodiversity is due to be held in T...
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Zielinski, C., on behalf of the authorship group listed below Tags: Open access, ARD Editorials Source Type: research
Lessons from negative phase 3 trials in rheumatoid arthritis anno 2023
A recent phase 3 trial (contRAst 3) with otilimab, a drug targeting granulocyte-macrophage colony-stimulating factor (GM-CSF), was reported by Taylor et al to be negative in a population of patients with rheumatoid arthritis (RA) refractory to conventional synthetic(cs)DMARDs and advanced therapies, including biological(b)DMARDs and targeted synthetic(ts)DMARDs.1 The trial not only failed to meet its primary endpoint with a weekly subcutaneous (SC) dose of 150 mg otilimab compared with placebo on top of csDMARDs, but also did not demonstrate non-inferiority to the active comparator sarilumab. Two other phase 3 trials compa...
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Westhovens, R., Verschueren, P. Tags: ARD Editorials Source Type: research
Correspondence on 'NCF1-339 polymorphism is associated with altered formation of neutrophil extracellular traps, high serum interferon activity and antiphospholipid syndrome in systemic lupus erythematosus by Linge et al
We read with great interest the article by Linge et al1 on ‘NCF1- 339 polymorphism is associated with altered formation of neutrophil extracellular traps, high serum interferon activity and antiphospholipid syndrome in systemic lupus erythematosus’1 as well as the response of Linge and Bengtsson2 to the comment by Joob and Wiwanitkit,3 who raised a question regarding the importance and the role of the NCF1-339 rs201802880 (p. Arg90His) polymorphism in systemic lupus erythematosus (SLE). Linge and Bengtsson3 nicely placed this polymorphism in a functional context, pointing out that the amino acid shift from argi...
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Goulielmos, G. N., Zervou, M. I., Eliopoulos, E. Tags: ARD Correspondence Source Type: research
Correspondence on 'The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease by Wallace et al
IgG4-related disease (IgG4-RD) was initially discovered as a clearly different entity in patients with sclerosing cholangitis with elevated levels of serum IgG4.1 Subsequently, the term ‘IgG4-related disease’ was proposed, and many other organ system manifestations were then linked to the IgG4-related disease. The first widely accepted and used diagnostic criteria for IgG4-RD was established in 2011. The comprehensive diagnostic criteria for IgG4-RD consisted of three major domains: organ involvement showing characteristic diffused or localised swelling or masses; elevated serum IgG4 concentrations (>135 mg/...
Source: Annals of the Rheumatic Diseases - November 10, 2023 Category: Rheumatology Authors: Yardımcı, G. K., Yıldırım, B. E. S., Farisogulları, B., Kilic, L., Karadag, O. Tags: ARD Correspondence Source Type: research
