Review
Medicine, General & Internal
Kholoud Almaabdi, Zareen Ahmad, Sindhu R. Johnson
Summary: Systemic sclerosis is an autoimmune disease characterized by immune abnormalities, vasculopathy, and fibrosis. Autoantibody testing, including ANA, anti-Scl-70, and anticentromere antibodies, has become increasingly important for diagnosis and prognostication. This review examines the epidemiology, clinical associations, and prognostic value of advanced autoantibody testing in systemic sclerosis.
Review
Rheumatology
Alba Navas-Otero, Sheila Gomez-De-Castro, Araceli Ortiz-Rubio, Alejandro Heredia-Ciuro, Javier Martin-Nunez, Andres Calvache-Mateo, Marie Carmen Valenza
Summary: This systematic review suggests that non-pharmacological interventions can improve hand function and performance of daily activities in individuals with systemic sclerosis. A meta-analysis of 8 studies showed that non-pharmacological interventions had better effects compared to waiting list or no treatment control conditions, but there was a moderate risk of bias.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2023)
Article
Immunology
Patrick Coit, Kaila L. Schollaert, Emily M. Mirizio, Kathryn S. Torok, Amr H. Sawalha
Summary: The study assessed DNA methylation differences between juvenile systemic sclerosis (jSSc) and juvenile localized scleroderma (jLS), revealing distinct epigenetic patterns and unique methylated genes in both diseases. Compared to healthy controls, differential methylation sites and genes were identified in jSSc and jLS, indicating potential novel diagnostic biomarkers for localized scleroderma. Pathway analysis showed enrichment of inflammatory pathways in jSSc and the HIPPO signaling pathway in jLS, highlighting different biological processes involved in each condition.
CLINICAL IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Mahmoud Nassar, Victoria Ghernautan, Nso Nso, Akwe Nyabera, Francisco Cuevas Castillo, Wan Tu, Luis Medina, Camelia Ciobanu, Mostafa Alfishawy, Vincent Rizzo, Saphwat Eskaros, Mamdouh Mahdi, Mohamed Khalifa, Mohamed El-Kassas
Summary: The gastrointestinal tract is frequently affected in individuals with systemic/localized scleroderma, leading to various GI manifestations and complications. Females are more susceptible to developing scleroderma, and esophageal and intestinal symptoms are common. Abnormal bowel movement and bacterial overgrowth contribute to malabsorption and increased mortality rate. Fecal incontinence is a common symptom that can lead to depression.
Article
Rheumatology
Robyn T. Domsic, Shiyao Gao, Maureen Laffoon, Steven Wisniewski, Yuqing Zhang, Virginia Steen, Robert Lafyatis, Thomas A. Medsger
Summary: Regardless of whether the first SSc manifestation or first non-Raynaud manifestation is used to define disease onset, a disease duration of less than 18 months at enrollment is preferable. A longer disease duration criterion more frequently results in regression to the mean of the mRSS score, which likely contributes to negative trial outcomes.
Review
Health Care Sciences & Services
Francesco Reggiani, Gabriella Moroni, Claudio Ponticelli
Summary: Systemic sclerosis is a chronic autoimmune disease that often involves the kidneys. Scleroderma renal crisis is the most serious renal event associated with this condition, and early and aggressive treatment is necessary to prevent irreversible organ damage and death.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
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)
Article
Rheumatology
Jamie Bering, W. Leroy Griffing, Michael Crowell, Sarah B. Umar
Summary: Research shows that patients with SSc generally demonstrate longitudinal stability or improvement in GI symptoms, but a subset of patients experience worsening of GI symptoms, leading to negative impacts on GI-related quality of life.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Immunology
Fatemeh Vafashoar, Kazem Mousavizadeh, Hadi Poormoghim, Amir Haghighi, Salar Pashangzadeh, Nazanin Mojtabavi
Summary: The study found that progesterone treatment increased collagen content in fibrotic and normal lung tissues, as well as increased alpha-SMA and TGF-beta in fibrotic lung tissues while decreasing MMP9. Furthermore, progesterone treatment also decreased the gene expression of Col1a2, Ctgf, and End1 in bleomycin-injured lung tissues.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Serena Fineschi
Summary: COVID-19 infection can trigger autoimmune diseases like systemic sclerosis (SSc) and lead to the production of autoantibodies. This case report describes a patient who developed autoantibody production and symptoms affecting the skin and gastrointestinal system after mild COVID-19 infection, eventually being diagnosed with SSc.
FRONTIERS IN IMMUNOLOGY
(2021)
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)
Review
Immunology
Maheswari Muruganandam, Angie Ariza-Hutchinson, Rosemina A. Patel, Wilmer L. Sibbitt
Summary: Systemic sclerosis is a complex autoimmune disease characterized by vascular damage, inflammation, and fibrosis. Biomarkers play an important role in understanding the disease process and potential therapeutic targets. Anti-nuclear antibodies are classical biomarkers, while other proteins and pathways are also implicated. The use of biomarker panels combined with advanced analysis techniques can help determine disease activity and treatment response.
JOURNAL OF INFLAMMATION RESEARCH
(2023)
Review
Rheumatology
Michael Hughes, Bashar Kahaleh, Christopher P. Denton, Justin C. Mason, Marco Matucci-Cerinic
Summary: In patients with SSc, the presence of SSc-AAV is associated with a severe disease course, typically presenting with features of microscopic polyangiitis or renal-limited vasculitis. The interaction between SSc and AAV exacerbates vascular disease, with similar patterns of major organ involvement. Current management strategies are based on treating SSc-AAV similar to isolated AAV, highlighting the need for further investigation and therapeutic strategies specific to this rare clinical entity.
Review
Chemistry, Medicinal
Barbara Ruaro, Marco Confalonieri, Marco Matucci-Cerinic, Francesco Salton, Paola Confalonieri, Mario Santagiuliana, Gloria Maria Citton, Elisa Baratella, Cosimo Bruni
Summary: Systemic sclerosis patients often suffer from interstitial lung disease, with early diagnosis playing a pivotal role in improving prognosis. Treatment strategies typically involve a combination of immunosuppressants and targeted biological therapies based on disease severity and progression risk. Hematopoietic autologous stem cell transplantation has shown benefits for progressive SSc patients, while lung transplantation is considered for refractory cases of SSc-ILD.
Review
Cardiac & Cardiovascular Systems
Peter Glynn, Sarah Hale, Tasmeen Hussain, Benjamin H. Freed
Summary: Systemic sclerosis (SSc) is a complex connective tissue disease that can affect various structural components of the heart, often being clinically silent in early disease. A review of cardiac imaging modalities emphasizes the importance of early detection and treatment optimization in SSc patients.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
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
Health Care Sciences & Services
Karolin R. Krause, Joanne Tay, William A. Douglas, Adrian Sammy, Ami Baba, Katherine Goren, Brett D. Thombs, Alison H. Howie, Maryam Oskoui, Ole Frobert, Yannis Trakadis, Julian Little, Beth K. Potter, Nancy J. Butcher, Martin Offringa
Summary: This article provides insights for future Registry-based randomized controlled trials (RRCTs) by analyzing the strengths and limitations reported in planned and completed RRCTs. The study identified six main RRCT strengths and four main RRCT limitations, and formulated ten recommendations for future RRCTs.
JOURNAL OF CLINICAL EPIDEMIOLOGY
(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
Rheumatology
Chelsea S. Rapoport, Alyssa Choi, Linda S. Kwakkenbos, Marie-Eve Carrier, Richard C. Henry, Luc D. Mouthon, Scott L. Roesch, Brett Thombs, Vanessa Malcarne
Summary: This study examined and compared the psychometric properties of the English and French versions of the University of California, Los Angeles, Loneliness Scale-6 (ULS-6) in patients with systemic sclerosis (SSc). The results showed that ULS-6 had acceptable reliability and validity in both English and French-speaking adults with SSc.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Multidisciplinary Sciences
Elsa-Lynn Nassar, Dalal A. Abdulkareem, Brett D. Thombs
Summary: This study aimed to synthesize evidence on the prevalence of mood and anxiety disorders and factors associated with symptoms in systemic sclerosis (SSc). Six eligible studies were identified, and the results showed that the prevalence of current or 30-day major depressive disorder varied from 4% to 29% across different populations. The prevalence of any anxiety disorder ranged from 49% to 51%. The study also found that higher education and marital status were associated with lower depressive symptoms, while factors associated with anxiety symptoms were not identified.
SCIENTIFIC REPORTS
(2023)
Article
Medicine, General & Internal
Quentin Kirren, Camille Daste, Frantz Foissac, Hendy Abdoul, Sophie Alami, Marie-Eve Carrier, Linda Kwakkenbos, Marie-Martine Lefevre-Colau, Francois Rannou, Agathe Papelard, Alexandra Roren, Brett D. Thombs, Luc Mouthon, Christelle Nguyen
Summary: This study aimed to explore differences in disability perception between patients and care providers in systemic sclerosis (SSc) through a cross-sectional internet-based mirror survey. Results showed that care providers' rates were higher than those of patients in all domains of the ICF-65 questionnaire. This difference was associated with care providers' characteristics, including organ-based specialty, younger age, and following patients with longer disease duration.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Multidisciplinary Sciences
Erica Wennberg, Sarah B. Windle, Kristian B. Filion, Brett D. Thombs, Genevieve Gore, Andrea Benedetti, Roland Grad, Carolyn Ells, Mark J. Eisenberg
Summary: With the increasing legalization of recreational cannabis in many countries, it is crucial to have roadside screening programs to detect and deter driving under the influence of cannabis. This systematic review assessed the effectiveness of roadside screening tests for cannabis use. The review included 101 studies and found that oral fluid tests showed the most promise for detecting blood THC levels over legal limits, while urine tests were generally not recommended and observational tests needed improvements in sensitivity.
Article
Rheumatology
Sabrina Provencher, Richard S. Henry, Carolina Bacalao, Marie-Eve Carrier, Linda Kwakkenbos, Brett D. Thombs
Summary: This study investigated whether the informational and support needs of people with SSc differ based on the time since their diagnosis. The findings revealed that individuals who were recently diagnosed with systemic sclerosis had higher needs in terms of discussing medical care as well as other aspects such as spirituality, talking with family and friends, financial issues, and sexual issues.
JOURNAL OF SCLERODERMA AND RELATED DISORDERS
(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)
