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
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)
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
Abeer Ghuman, Dinesh Khanna, Celia J. F. Lin, Daniel E. Furst, Ganesh Raghu, Fernando J. Martinez, Mauro Zucchetto, Suiyuan Huang, Angus Jennings, Svetlana Nihtyanova, Christopher P. Denton
Summary: This study investigated prognostic and predictive markers for SSc-associated interstitial lung disease (SSc-ILD). The results showed that male patients were more likely to experience a decline in lung function. In the anti-inflammatory treatment, sex, early disease duration, IL-6 levels, and anti-topoisomerase antibodies were predictive factors for reducing lung function decline.
Article
Rheumatology
Brittany L. Adler, Francesco Boin, Paul J. Wolters, Clifton O. Bingham, Ami A. Shah, Carol Greider, Livia Casciola-Rosen, Antony Rosen
Summary: Autoantibodies targeting telomere-associated proteins are found in a subset of patients with systemic sclerosis (SSc), and are associated with short lymphocyte telomere length and lung disease. These autoantibodies are rarely present in rheumatoid arthritis, myositis, and healthy controls, suggesting a specificity for SSc and idiopathic pulmonary fibrosis (IPF).
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Review
Biochemistry & Molecular Biology
Bianca Saveria Fioretto, Irene Rosa, Marco Matucci-Cerinic, Eloisa Romano, Mirko Manetti
Summary: Systemic sclerosis (SSc, scleroderma) is a rare connective tissue disease characterized by immune dysregulation, small vessel vasculopathy, impaired angiogenesis, and fibrosis. Microvascular impairment is the initial event of the disease and is linked to disabling and life-threatening clinical manifestations. Despite available treatment options, managing SSc-related vascular disease remains challenging. Vascular biomarkers have shown promise in assessing disease progression, predicting prognosis, and guiding therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Kristina Elizabeth Neergaard Clark, Shiwen Xu, Moustafa Attah, Voon H. Ong, Christopher Dominic Buckley, Christopher P. Denton
Summary: This study investigated cellular differences in diffuse cutaneous systemic sclerosis (dcSSc) patients compared with healthy controls (HCs), and found differences in cell clusters between the ARA+ and ATA+ subsets, highlighting the importance of considering antibodies and disease stage in the management and trial design of dcSSc.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Rheumatology
Leonardo Martin Calderon, Mitali Chaudhury, Janet E. Pope
Summary: This study provides a systematic review of the economic impact and healthcare resource utilization associated with systemic sclerosis (SSc). The findings indicate that SSc poses a significant economic burden on patients and healthcare resources, with the burden increasing with SSc-associated complications and varying depending on geographical location and access to healthcare resources.
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.
Article
Rheumatology
Sophie I. E. Liem, Sam Neppelenbroek, Cynthia M. Fehres, Brigitte A. Wevers, Rene E. M. Toes, Cornelia F. Allaart, Tom W. J. Huizinga, Hans Ulrich Scherer, Jeska K. De Vries-Bouwstra
Summary: Early diagnosis of systemic sclerosis (SSc) is important. The most frequent SSc-specific autoantibodies are anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (ATA). ATA is associated with severe disease course. More detailed characterisation of the ATA-response in SSc might improve prognostication.
Review
Biology
Michele Colaci, Maria Letizia Aprile, Domenico Sambataro, Gianluca Sambataro, Lorenzo Malatino
Summary: The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the possible coexistence of other autoimmune hepatic disorders. However, the occurrence of portal hypertension and, more specifically, of the syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature for SSc patients. No specific SSc pattern linked to IPH emerged, even though reports from the literature often described the limited skin subset. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. Besides being a rare condition, the onset of IPH in SSc patients is an occurrence that should be taken into account.
Review
Rheumatology
Tze Chin Tan, Maria Noviani, Ying Ying Leung, Andrea Hsiu Ling Low
Summary: Systemic sclerosis (SSc) is a disease characterized by immune dysregulation, vasculopathy, and fibrosis of multiple organs, with the gastrointestinal (GI) tract being the most commonly affected organ. Recent studies have found unique microbial taxa alterations in the GI microbiome of SSc patients compared to healthy controls (HC), with potential associations with disease manifestations and severity.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
Article
Rheumatology
Christopher P. Denton, Shiwen Xu, Fenge Zhang, Rory H. Maclean, Kristina E. N. Clark, Signe Borchert, Rizwan Hussain, Joerg Klingelhofer, Jonas Hallen, Voon H. Ong
Summary: This study investigated the role of S100A4 in fibroblast activation in systemic sclerosis (SSc). The results showed that S100A4 concentration was higher in SSc patients compared to healthy controls, and was associated with interstitial lung disease and scleroderma renal crisis. In vitro experiments demonstrated that S100A4 promoted the expression of profibrotic genes in SSc fibroblasts, while a neutralizing antibody against S100A4 reduced this expression. These findings provide evidence for the profibrotic role of S100A4 in SSc and suggest its potential as a therapeutic target.
ANNALS OF THE 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)
Article
Medicine, General & Internal
Yassmin Khadra, Jennifer G. Walker, Paul Hakendorf, Susanna Proudman, Maureen Rischmueller, Catherine L. Hill, Samuel Whittle, Eliza Pontifex, Peter J. Roberts-Thomson
Summary: SRC is a rare but severe complication with a stable incidence of 0.58/million/year in South Australia. Patients have poor survival outcomes, often requiring renal replacement therapy and transplantation. Nailfold capillaroscopy reveals extensive microvascular damage. No improvement in survival was observed over the 34-year study period.
INTERNAL MEDICINE JOURNAL
(2021)
Article
Rheumatology
Jessica L. Fairley, Dylan Hansen, Susanna Proudman, Joanne Sahhar, Gene-Siew Ngian, Jenny Walker, Gemma Strickland, Michelle Wilson, Kathleen Morrisroe, Nava Ferdowsi, Gabor Major, Janet Roddy, Wendy Stevens, Mandana Nikpour
Summary: This study describes the clinical characteristics and outcomes of patients with SSc-MCTD, SSc overlap, and SSc only, revealing that anti-RNP antibodies are associated with better survival.
ARTHRITIS CARE & RESEARCH
(2021)
Article
Rheumatology
Laura Ross, Wendy Stevens, Michelle Wilson, Molla Huq, Gemma Strickland, Jennifer Walker, Joanne Sahhar, Gene-Siew Ngian, Janet Roddy, Peter Youssef, Susanna Proudman, Mandana Nikpour
CLINICAL RHEUMATOLOGY
(2020)
Article
Oncology
William Murray-Brown, Tom D. Wilsdon, Helen Weedon, Susanna Proudman, Shawgi Sukumaran, Sonja Klebe, Jennifer G. Walker, Malcolm D. Smith, Mihir D. Wechalekar
JOURNAL FOR IMMUNOTHERAPY OF CANCER
(2020)
Article
Rheumatology
Shreeya Patel, Kathleen Morrisroe, Susanna Proudman, Dylan Hansen, Joanne Sahhar, Malcolm R. Sim, Gene-Siew Ngian, Jenny Walker, Gemma Strickland, Michelle Wilson, Nava Ferdowsi, Gabor Major, Janet Roddy, Wendy Stevens, Mandana Nikpour
Article
Cardiac & Cardiovascular Systems
Karthigesh Sree Raman, Ranjit Shah, Michael Stokes, Angela Walls, Richard J. Woodman, Rajiv Ananthakrishna, Jennifer G. Walker, Susanna Proudman, Peter M. Steele, Carmine G. De Pasquale, David S. Celermajer, Joseph B. Selvanayagam
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2020)
Article
Rheumatology
A. Y. S. Lee, K. A. Patterson, D. J. Tan, M. E. Wilson, S. M. Proudman, W. Stevens, M. Nikpour, J. Sahhar, G-S Ngian, J. Roddy, P. J. Roberts-Thomson, J. G. Walker
Summary: The study found that anti-Ro52/TRIM21 was independently associated with pulmonary arterial hypertension (PAH) and increased mortality risk in systemic sclerosis (SSc) patients, but not with interstitial lung disease (ILD) or other indicators of pulmonary involvement. Future longitudinal studies are recommended to investigate the timing and pathogenic mechanisms of this autoantibody in PAH.
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
An Tran-Duy, Kathleen Morrisroe, Philip Clarke, Wendy Stevens, Susanna Proudman, Joanne Sahhar, Mandana Nikpour
Summary: The cost-effectiveness of combination therapy for systemic sclerosis-related pulmonary arterial hypertension was evaluated. The results showed that combination therapy was more cost-effective compared to monotherapy, especially in terms of quality-adjusted life years. At higher willingness-to-pay thresholds, combination therapy was considered a cost-effective option.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2021)
Article
Medicine, General & Internal
Vandana Bhushan, Susan Lester, Liz Briggs, Raif Hijjawi, E. Michael Shanahan, Eliza Pontifex, Jem Ninan, Catherine Hill, Fin Cai, Jennifer Walker, Fiona Goldblatt, Mihir D. Wechalekar
Summary: Similar retention rates of the second and third compared to the first b/tsDMARD in RA, PsA, and AS support a differential b/tsDMARDs use strategy based on patient presentation. The risk of b/tsDMARD failure was halved in PsA patients, and covariates were not associated with the risk of bDMARD failure. The most common reason for switching was treatment failure.
FRONTIERS IN MEDICINE
(2021)
Article
Behavioral Sciences
Lucie Burnod, Karen Patterson, Kacie Dickinson, John Coveney
Summary: The father's role in household responsibilities has changed over the past fifty years, with expectations for them to be more involved in family food provisioning. However, they are often still seen as supplementary to the mother's role. This qualitative study explores the beliefs and expectations of both mothers and fathers regarding the father's role in family food provisioning. The findings reveal a discrepancy between the fathers' attitudes and behaviors, with the majority of mothers desiring a more equal share but not necessarily expecting it to be met.
Article
Behavioral Sciences
Georgia Middleton, Rebecca K. Golley, Karen A. Patterson, John Coveney
Summary: This research used grounded theory to analyze the components required for successfully executing a family meal. The study found that the success of a family meal is closely related to cognition, actions, outcomes, beliefs and feelings, and the person responsible. This new understanding provides important intervention opportunities for family meal research and practice.
Article
Sociology
Georgia Middleton, Stefania Velardo, Karen A. Patterson, John Coveney
Summary: Attending lunch clubs is important for social interaction and can help reduce social isolation and loneliness among older culturally and linguistically diverse immigrants. This study reveals the positive impact of lunch clubs on the health and wellbeing of attendees.
FOOD CULTURE & SOCIETY
(2022)
Review
Medicine, General & Internal
Peter J. Roberts-Thomson, Karen A. Patterson, Jennifer G. Walker
Summary: Nailfold capillaroscopy (NFC) is a simple noninvasive microscopic technique used to identify abnormal morphological changes in the nailfold capillaries. It plays a fundamental role in understanding the pathological processes of scleroderma spectrum disorders, and can be performed using ophthalmoscope, dermatoscope, or smart phone for diagnosis. NFC has the potential to be a biomarker in the management of scleroderma spectrum disorders.
INTERNAL MEDICINE JOURNAL
(2023)
Letter
Pathology
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