Article
Rheumatology
Kristina Elizabeth Neergaard Clark, Corrado Campochiaro, Eszter Csomor, Adam Taylor, Katherine Nevin, Nicholas Galwey, Mary A. Morse, Jennifer Singh, Yee Voan Teo, Voon H. Ong, Emma Derrett-Smith, Nicolas Wisniacki, Shaun M. Flint, Christopher P. Denton
Summary: Clinical heterogeneity is a key feature of systemic sclerosis (SSc), with hallmark SSc autoantibodies playing a central role in diagnosis and associating with distinct patterns of complications. This study used high-dimensional transcriptional and proteomic analysis to reveal a molecular spectrum of SSc based on skin gene expression and serum protein analysis, showing differences in fibrosis markers and gene expression profiles between different autoantibody specificities. Individual patient pathway analysis identified overlapping and distinct disease processes within SSc subgroups.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Rheumatology
Sabrina Hoa, Selma Lazizi, Murray Baron, Mianbo Wang, Marvin J. Fritzler, Marie Hudson
Summary: There is an association between cancer and specific autoantibodies in SSc patients, with increased cancer risk in patients positive for anti-topoisomerase I and anti-U1-RNP antibodies. However, synchronous cancer is rare in this cohort of Canadian SSc patients.
Article
Biology
Sevdalina Nikolova Lambova, Ekaterina Krasimirova Kurteva, Sanie Syuleymanova Dzhambazova, Georgi Hristov Vasilev, Dobroslav Stanimirov Kyurkchiev, Mariela Gencheva Geneva-Popova
Summary: In this pilot study involving 19 patients with SSc, a correlation between more advanced capillaroscopic changes and the presence of anti-Scl-70 autoantibodies was confirmed. Additionally, positive anti-RNAP III-155 antibodies were found in SSc patients with or without early microangiopathy.
Article
Rheumatology
Burcu Ayoglu, Michele Donato, Daniel E. Furst, Leslie J. Crofford, Ellen Goldmuntz, Lynette Keyes-Elstein, Judith James, Susan Macwana, Maureen D. Mayes, Peter McSweeney, Richard A. Nash, Keith M. Sullivan, Beverly Welch, Ashley Pinckney, Rong Mao, Lorinda Chung, Purvesh Khatri, Paul J. Utz
Summary: Results from the SCOT clinical trial showed that HSCT had significant benefits over CTX in patients with systemic sclerosis. The objective of this study was to test the hypothesis that transplantation stabilizes the autoantibody repertoire in patients with favorable clinical outcomes. Analysis of autoantibody profiles revealed significant differences between HSCT and CTX-treated patients, suggesting that HSCT alters the autoantibody repertoire while CTX treatment does not.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Immunology
Claire F. Beesley, Nina R. Goldman, Taher E. Taher, Christopher P. Denton, David J. Abraham, Rizgar A. Mageed, Voon H. Ong
Summary: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by excessive extracellular matrix deposition. B cells play a fundamental role in the pathogenesis and development of SSc, as they infiltrate lesional sites and produce profibrotic cytokines. B cell counts are increased in SSc patients and show differences in various B cell compartments. B cell signaling is impaired in SSc patients, and B cell depletion therapy has shown therapeutic benefits.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Elopy N. Sibanda, Yvonne Dube, Mazvita Chakawa, Takafira Mduluza, Francisca Mutapi
Summary: This study investigated the clinical and laboratory characteristics of Zimbabwean patients with SSc specific autoantibodies, revealing racial variations in the expression of these antibodies. Most patients did not meet the classification criteria for SSc but still exhibited significant symptoms. Respiratory and skin symptoms were among the most common in the patients.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Jean -Baptiste Vulsteke, Vanessa Smith, Carolien Bonroy, Rita Derua, Daniel Blockmans, Petra De Haes, Steven Vanderschueren, Jan L. Lenaerts, Kristl G. Claeys, Wim A. Wuyts, Patrick Verschueren, Gilles Vanhandsaeme, Yves Piette, Ellen De Langhe, Xavier Bossuyt
Summary: The study identified 8 telomere-and telomerase-associated autoantigens in SSc patients using unlabeled protein immunoprecipitation combined with gel-free liquid chromatography-tandem mass spectrometry. These findings provide new insights into the pathogenesis of SSc.
JOURNAL OF AUTOIMMUNITY
(2023)
Review
Biochemistry & Molecular Biology
Hanna Grasshoff, Konstantinos Fourlakis, Sara Comduhr, Gabriela Riemekasten
Summary: Systemic sclerosis is a rare connective tissue disorder characterized by immune dysregulation, inflammation, fibrosis and vasculopathy. Alterations in the B-cell compartment, including polyclonal B-cell hyperreactivity and autoantibody production, are important in the pathogenesis of the disease. Autoantibodies serve as biomarkers and contribute to pathological mechanisms. Therapeutic approaches targeting B cells and autoantibodies may hold promise in the treatment of systemic sclerosis.
Article
Allergy
Gemma Lepri, Martina Catalano, Silvia Bellando-Randone, Serena Pillozzi, Elisa Giommoni, Roberta Giorgione, Cristina Botteri, Marco Matucci-Cerinic, Lorenzo Antonuzzo, Serena Guiducci
Summary: There is a well-known association between systemic sclerosis (SSc) and cancer, suggesting shared genetic and environmental risk factors. The use of immunosuppressive drugs in SSc may increase the risk of cancer occurrence, and certain serological features such as anti-RNA polymerase III and anti-topoisomerase I autoantibodies appear to be associated with increased cancer frequency in SSc patients. Additionally, lung fibrosis, long-standing SSc pulmonary involvement, and specific medication exposures have been linked to different types of cancer.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Pawel Zebryk, Piotr Przymuszala, Jan Krzysztof Nowak, Tomasz Piorunek, Tatiana Mularek-Kubzdela, Mariusz Puszczewicz
Summary: This study evaluated the prevalence and clinical significance of SSc-related autoantibodies and compared the sensitivity of two line immunoblot assays. The findings indicate that different autoantibodies are associated with specific characteristics of SSc.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Immunology
Aurelien Chepy, Solange Vivier, Fabrice Bray, Camille Ternynck, Jean-Pascal Meneboo, Martin Figeac, Alexandre Filiot, Lucile Guilbert, Manel Jendoubi, Christian Rolando, David Launay, Sylvain Dubucquoi, Guillemette Marot, Vincent Sobanski
Summary: This study investigated the effect of purified immunoglobulin G (IgG) from systemic sclerosis (SSc) patients on protein and mRNA expression of dermal fibroblasts (FBs) using a multi-omics approach. The results showed that SSc sera and purified IgG induced unique protein profile patterns, and this effect depended on the serotype of the patient.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Fabian A. Mendoza, Anthony DiMarino, Sidney Cohen, Christopher Adkins, Shady Abdelbaki, Satish Rattan, Christopher Cao, Susie Denuna-Rivera, Sergio A. Jimenez
Summary: This article describes the successful treatment of two patients with SSc-associated swallowing dysfunction using IVIG, resulting in significant symptom improvement and improved esophageal motility.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medicine, General & Internal
Mariko Hara, Rumi Ueha, Taku Sato, Takao Goto, Ayumi Yoshizaki, Hayakazu Sumida, Shinichi Sato, Tatsuya Yamasoba
Summary: Systemic sclerosis is often associated with dysphagia and esophageal dysmotility. This study evaluated the association between dysphagia and esophageal dysmotility in patients with systemic sclerosis and identified risk factors. The results showed that dysphagia was associated with autoantibody positivity and older age, while there were no identified risk factors for esophageal dysmotility. No correlation was found between dysphagia and esophageal dysmotility.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Rheumatology
Lea Lopez, Thomas Barnetche, Gael Galli, Julien Seneschal, Elodie Blanchard, Emilie Shipley, Jean-Luc Pellegrin, Estibaliz Lazaro, Joel Constans, Pierre Duffau, Thierry Schaeverbeke, Christophe Richez, Marie Kostine, Marie-Elise Truchetet
Summary: This study observed an increased risk of cancer in systemic sclerosis (SSc) patients with RNA polymerase III antibodies. Other risk factors identified were age, time of SSc diagnosis, interstitial lung disease, and anti-topoisomerase antibodies. Regular cancer screening should be considered for these patients.
Article
Pharmacology & Pharmacy
Rong-Hong Guo, Hao Cheng, Xiao-Ying Zhang, Zhen Yu, Guang-Hui Wang, Shu-Ya Hao, Xiao-Peng Gao, Hong-Yan Wen
Summary: This study aimed to investigate the changes in absolute numbers of T-lymphocyte subsets and serum cytokines in patients with systemic sclerosis (SSc). The study found that patients with SSc had lower numbers of Th2 and Treg cells compared to healthy controls, which led to significantly increased ratios of Th1/Th2 and Th17/Treg. Additionally, the levels of certain cytokines were significantly higher in SSc patients. The findings contribute to a better understanding of the pathogenesis of SSc and provide a basis for the development of novel therapeutic interventions targeting these cells and cytokines.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Rheumatology
Ariane Barbacki, Murray Baron, Mianbo Wang, Yuqing Zhang, Wendy Stevens, Joanne Sahhar, Susanna Proudman, Mandana Nikpour, Ada Man
Summary: This study found distinct trajectories of damage accrual in patients with systemic sclerosis, with age, gender, disease type, tendon friction rubs, and C-reactive protein level being associated with the different trajectories. These findings may be helpful in identifying patients who require early aggressive treatment.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Tatiana Nevskaya, Leonardo M. Calderon, Murray Baron, Janet E. Pope
Summary: Digital ulcers in systemic sclerosis patients lead to increased utilization of healthcare resources, including tests, visits, and aids. Even after adjusting for disease severity in other organ systems, the presence of digital ulcers remains a significant predictor of more frequent physician visits and more tests.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Kiem Oen, Karine Toupin-April, Brian M. Feldman, Roberta A. Berard, Cia'ran M. Duffy, Lori B. Tucker, Jiahao Tian, Dax G. Rumsey, Jaime Guzman
Summary: This study validates the JIA parent global assessment as a valid measure of health-related quality of life. Comparisons with other HRQoL measures show good construct validity. However, reliability estimates and measurement errors were unsatisfactory, likely due to the time interval between assessments. Causal pathway analysis confirms previous findings.
Article
Rheumatology
Siobhan Deshauer, Mats Junek, Murray Baron, Karen A. Beattie, Margaret J. Larche
Summary: This study examined the impact of pregnancy on disease activity in women with systemic sclerosis. The results showed that having at least one pregnancy after diagnosis did not significantly affect long-term renal, respiratory, or global function outcomes. However, postpartum complications should still be monitored.
JOURNAL OF SCLERODERMA AND RELATED DISORDERS
(2023)
Review
Rheumatology
Andreas Kerschbaumer, Alexandre Sepriano, Sytske Anne Bergstra, Josef S. Smolen, Desiree van der Heijde, Roberto Caporali, Christopher John Edwards, Patrick Verschueren, Savia de Souza, Janet E. Pope, Tsutomu Takeuchi, Kimme L. Hyrich, Kevin L. Winthrop, Daniel Aletaha, Tanja A. Stamm, Jan W. Schoones, Robert B. M. Landewe
Summary: This study aimed to update the evidence on the efficacy of DMARDs and provide information for the 2022 update of EULAR recommendations for RA management. A systematic literature review was conducted to investigate the efficacy of cs, b, biosimilar, and tsDMARDs in RA patients. The results showed that csDMARDs combined with short-term glucocorticoids in early RA were as effective as bDMARD+MTX combination therapy. JAK inhibitors were effective in different patient populations. Tapering of DMARDs was feasible for some patients who could maintain low disease activity or achieve remission.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Editorial Material
Rheumatology
Janet E. Pope, Roy M. Fleischmann
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Josef S. Smolen, Robert B. M. Landewe, Sytske Anne Bergstra, Andreas Kerschbaumer, Alexandre Sepriano, Daniel Aletaha, Roberto Caporali, Christopher John Edwards, Kimme L. Hyrich, Janet E. Pope, Savia de Souza, Tanja A. Stamm, Tsutomu Takeuchi, Patrick Verschueren, Kevin L. Winthrop, Alejandro Balsa, Joan M. Bathon, Maya H. Buch, Gerd R. Burmester, Frank Buttgereit, Mario Humberto Cardiel, Katerina Chatzidionysiou, Catalin Codreanu, Maurizio Cutolo, Alfons A. den Broeder, Khadija El Aoufy, Axel Finckh, Joao Eurico Fonseca, Jacques-Eric Gottenberg, Espen A. Haavardsholm, Annamaria Iagnocco, Kim Lauper, Zhanguo Li, Iain B. McInnes, Eduardo F. Mysler, Peter Nash, Gyula Poor, Gorica G. Ristic, Felice Rivellese, Andrea Rubbert-Roth, Hendrik Schulze-Koops, Nikolay Stoilov, Anja Strangfeld, Annette van der Helm-van Mil, Elsa van Duuren, Theodora P. M. Vliet Vlieland, Rene Westhovens, Desiree van der Heijde
Summary: This study provides an update on the management recommendations for rheumatoid arthritis (RA) based on the latest developments in the field. The recommendations cover various aspects including medication use, combination therapy, treatment strategies, and dose reduction. The consensus was reached on 5 overarching principles and 11 specific recommendations.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Rheumatology
Alexandre Sepriano, Andreas Kerschbaumer, Sytske Anne Bergstra, Josef S. Smolen, Desiree van der Heijde, Roberto Caporali, Christopher J. Edwards, Patrick Verschueren, Savia de Souza, Janet Pope, Tsutomu Takeuchi, Kimme Hyrich, Kevin L. Winthrop, Daniel Aletaha, Tanja Stamm, Jan W. Schoones, Robert B. M. Landewe
Summary: This study conducted a systematic literature review (SLR) to evaluate the safety of synthetic and biological disease-modifying antirheumatic drugs (DMARDs) for the management of rheumatoid arthritis (RA). The results indicated that bDMARDs were associated with an increased risk of serious infections compared to csDMARDs, while JAK inhibitors (JAKi) showed higher risk of herpes zoster infections. The risks of malignancies, major adverse cardiovascular events (MACE), and venous thromboembolism (VTE) were similar among different treatments.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Rheumatology
Sytske Anne Bergstra, Alexandre Sepriano, Andreas Kerschbaumer, Desiree van der Heijde, Roberto Caporali, Christopher John Edwards, Patrick Verschueren, Savia de Souza, Janet E. Pope, Tsutomu Takeuchi, Kimme L. Hyrich, Kevin L. Winthrop, Daniel Aletaha, Tanja A. Stamm, Jan W. Schoones, Josef S. Smolen, Robert B. M. Landewe
Summary: This systematic literature review examines the efficacy, duration of use, and safety of glucocorticoids (GCs) in the management of rheumatoid arthritis (RA). The study confirms the effectiveness of GCs in treating RA, with most patients able to stop GCs within 12-24 months. However, the use of GCs also carries well-known safety risks, including osteoporotic fractures, serious infections, diabetes, and mortality.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Rehabilitation
Amanda Wurz, Delaney Duchek, Kelsey Ellis, Mannat Bansal, Marie-Eve Carrier, Lydia Tao, Laura Dyas, Linda Kwakkenbos, Brooke Levis, Ghassan El-Baalbaki, Danielle B. Rice, Yin Wu, Richard S. Henry, Laura Bustamante, Sami Harb, Shannon Hebblethwaite, Scott B. Patten, Susan J. Bartlett, John Varga, Luc Mouthon, Sarah Markham, Brett D. Thombs, S. Nicole Culos-Reed
Summary: This study explored the impact of the videoconference-based supportive care program (SPIN-CHAT program) on individuals with systemic sclerosis (SSc) during early COVID-19. Interviews with trial participants and research team members revealed that the program had positive effects on psychological health, provided SSc-specific support, created a safe space for group interaction, reduced boredom, and offered self-management skills.
DISABILITY AND REHABILITATION
(2023)
Review
Rheumatology
Janet E. E. Pope, Christopher P. P. Denton, Sindhu R. R. Johnson, Andreu Fernandez-Codina, Marie Hudson, Tatiana Nevskaya
Summary: Pope et al. review the current management of organ-based manifestations of systemic sclerosis, including screening and treatment. The treatment for systemic sclerosis has improved, with immunosuppressive agents and autologous haematopoietic stem cell transplantation showing promise. Therapies for interstitial lung disease and pulmonary arterial hypertension have also improved, with mycophenolate mofetil surpassing cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Research is needed to develop targeted and effective treatments for other manifestations and to establish best practices for screening and early intervention.
NATURE REVIEWS RHEUMATOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Charmaine van Eeden, Desiree Redmond, Naima Mohazab, Maggie J. J. Larche, Andrew L. L. Mason, Jan Willem Cohen Tervaert, Mohammed S. S. Osman
Summary: This study aimed to assess the prevalence of ME/CFS in early limited cutaneous systemic sclerosis (lcSSc) patients and determine if alterations in mitochondrial gene expression and mtDNA integrity could be used to distinguish between fatigued and non-fatigued patients. ME/CFS-related symptoms were assessed through questionnaires, and the expression of mitochondrial gene transcripts and mtDNA integrity were quantified. The study found that SSc patients with ME/CFS could be differentiated from non-fatigued patients through mitochondrial gene analysis, specifically with reduced expression of ND4 and CyB and increased expression of Cox7C. ND4 and CyB expression were also correlated with disease severity indicators.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Letter
Rheumatology
Glen S. Hazlewood, Pooneh Akhavan, Jordi Pardo Pardo, Arnav Agarwal, Orit Schieir, Claire E. H. Barber, Laurie Proulx, Dawn P. Richards, Claire Bombardier, Janet E. Pope, Cheryl Barnabe, Peter Tugwell, Shahin Jamal, J. Carter Thorne, Roko P. A. Nikolic, Majed Khraishi, Nick Bansback, Alexandra Legge, Vivian Bykerk, Regina Taylor-Gjevre
JOURNAL OF RHEUMATOLOGY
(2023)
Article
Rheumatology
Matthew Wong-Pack, Elliot Hepworth, Mohammad Movahedi, Bindee Kuriya, Janet Pope, Edward Keystone, Carter Thorne, Vandana Ahluwalia, Angela Cesta, Carol Mously, Claire Bombardier, Arthur Lau, Sibel Zehra Aydin
Summary: This study examined the impact of the COVID-19 pandemic on RA patients' outcomes and medication profiles, and found that disease activity and PROs remained stable during the pandemic, while there were changes in medication use.
RHEUMATOLOGY ADVANCES IN PRACTICE
(2023)
Article
Rheumatology
E. Rampakakis, C. Thorne, A. Cesta, M. Movahedi, X. Li, C. Mously, V. Ahluwalia, J. Brophy, P. Ciaschini, E. Keystone, A. Lau, G. Major, V. Pavlova, J. Pope, C. Bombardier
Summary: This study examines the prevalence of medical cannabis use and rheumatologists' perceptions among patients attending rheumatology clinics in Ontario. The results showed a high proportion of medical cannabis users, who were younger and had more severe symptoms compared to non-users. Pain, sleep disorders, and anxiety were the most commonly treated symptoms. Most rheumatologists reported being uncomfortable to authorize medical cannabis due to lack of evidence, knowledge, and product standardization.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Article
Rheumatology
Walter P. Maksymowych, Nele Herregods, Nisha Varma, Arthur B. Meyers, Jennifer Stimec, Andrea S. Doria, Nikolay Tzaribachev, Tarimobo M. Otobo, Marion A. van Rossum, Joel Paschke, Stephanie Wichuk, Robert G. Lambert
Summary: This study aimed to investigate whether systematic calibration improves the scoring proficiency of JAMRIS-SIJ and whether contrast-enhancement enhances its performance. The results showed that calibrated readers achieved greater reliability in scoring specific inflammatory and structural lesions. Sensitivity and reliability for scoring inflammatory lesions were higher on fluid-sensitive sequences compared to contrast-enhanced sequences. Therefore, systematic calibration should be implemented before using JAMRIS-SIJ in clinical trials, and it is unlikely that contrast-enhanced MRI will improve the performance of this method.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)
Article
Rheumatology
L. van Ouwerkerk, S. A. Bergstra, T. D. Maarseveen, T. W. J. Huizinga, R. Knevel, C. F. Allaart
Summary: This study evaluated whether the initial use of glucocorticoid (GC) bridging in RA patients leads to a higher probability of long-term GC and bDMARD use. The results showed that patients who initially started GC had a higher risk of later GC use, but the risk of bDMARD use was not significantly increased.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)
Article
Rheumatology
ShuangHua Liu, YiMei Tan, WeiDong Huang, HongSheng Luo, BingCheng Pan, Shuan Wu
Summary: This study assessed the cardiovascular safety of zoledronic acid in the treatment of primary osteoporosis. The results showed that in women with primary osteoporosis, zoledronic acid may increase the risk of atrial fibrillation and arrhythmias, but the cardiovascular risk in men with osteoporosis is uncertain.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)
