Article
Rheumatology
Benjamin E. Decato, Ron Ammar, Lauren Reinke-Breen, John R. Thompson, Anthony Azzara
Summary: This study explored the molecular basis for SSc pathogenesis in a mouse model of scleroderma, identifying changes in gene expression related to anti-fibrotic therapy and implementing a machine learning algorithm for predicting lung function using transcriptome data. The findings provide insights into SSc pathogenesis and intervention pathways, offering a rich dataset for future fibrotic disease research.
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
George Iliopoulos, Dimitrios Daoussis
Summary: Research has shown limited data about renal impairment in SSc beyond scleroderma renal crisis (SRC). However, studies indicate that renal dysfunction in SSc is not uncommon, with a significant percentage of patients having decreased glomerular filtration rate (GFR). The Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula is suggested as a suitable tool for assessing renal function in SSc patients.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Allergy
Alice Cole, Voon H. Ong, Christopher P. Denton
Summary: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a high mortality rate. Although angiotensin-converting enzyme inhibitor therapy has improved renal outcomes, there are currently no preventative measures and patients may rapidly decline. Studies have identified specific phenotypes at higher risk of developing SRC and novel urinary biomarkers may aid in early identification and treatment. Clear guidelines for management have been established to standardize care and promote collaboration between rheumatology and renal physicians. While outcomes following renal transplant have improved, further research is needed to understand the mechanisms and develop new therapies for SRC.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(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.
Article
Rheumatology
Hideaki Tsuji, Nobuo Kuramoto, Tsuneo Sasai, Mirei Shirakashi, Hideo Onizawa, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Ryu Watanabe, Akira Onishi, Kosaku Murakami, Hajime Yoshifuji, Masao Tanaka, Motomu Hashimoto, Koichiro Ohmura, Akio Morinobu
Summary: Specific SSc-related autoantibodies are associated with the morbidity and mortality of SRC. Anti-topoisomerase I, anti-RNAPIII, and anti-U1-RNP are associated with the development of SRC, while anti-topoisomerase I is associated with 1-year mortality of SRC.
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
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.
Article
Rheumatology
Jose B. Nergon, Maria A. Lopez-Olivo, Loreto Carmona, Robin Christensen, Francesca Ingegnoli, Natalia Zamora, Jorge I. Gamez-Nava, Laura Gonzalez-Lopez, Vibeke Strand, Niti Goel, Tiffany Westrich-Robertson, Maria E. Suarez-Almazor
Summary: This study aimed to identify patient-centered domains with long-term relevance to people with rheumatoid arthritis (RA). Semi-structured individual cognitive interviews were conducted with RA patients from five different countries. Six main themes were identified, including living with symptoms and functional limitations, lack of participation, partner and family issues, risk of damage to vital organs, coping strategies, and healthcare concerns. These domains are seldom measured in longitudinal registries and should be considered in patient-centered longitudinal studies.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2023)
Article
Rheumatology
David Roofeh, Kevin K. Brown, Ella A. Kazerooni, Donald Tashkin, Shervin Assassi, Fernando Martinez, Athol U. Wells, Ganesh Raghu, Christopher P. Denton, Lorinda Chung, Anna-Maria Hoffmann-Vold, Oliver Distler, Kerri A. Johannson, Yannick Allanore, Eric L. Matteson, Leticia Kawano-Dourado, John D. Pauling, James R. Seibold, Elizabeth R. Volkmann, Simon L. F. Walsh, Chester Oddis, Eric S. White, Shaney L. Barratt, Elana J. Bernstein, Robyn T. Domsic, Paul F. Dellaripa, Richard Conway, Ivan Rosas, Nitin Bhatt, Vivien Hsu, Francesca Ingegnoli, Bashar Kahaleh, Puneet Garcha, Nishant Gupta, Surabhi Khanna, Peter Korsten, Celia Lin, Stephen C. Mathai, Vibeke Strand, Tracy J. Doyle, Virginia Steen, Donald F. Zoz, Juan Ovalles-Bonilla, Ignasi Rodriguez-Pinto, Padmanabha D. Shenoy, Andrew Lewandoski, Elizabeth Belloli, Alain Lescoat, Vivek Nagaraja, Wen Ye, Suiyuan Huang, Toby Maher, Dinesh Khanna
Summary: This study aimed to establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). The results showed that using the proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time.
Article
Rheumatology
Karin Melsens, Maurizio Cutolo, Dieneke Schonenberg-Meinema, Ivan Foeldvari, Maria C. Leone, Yora Mostmans, Valerie Badot, Rolando Cimaz, Joke Dehoorne, Ellen Deschepper, Tracy Frech, Johanna Hernandez-Zapata, Francesca Ingegnoli, Archana Khan, Dorota Krasowska, Hartwig Lehmann, Ashima Makol, Miguel A. Mesa-Navas, Malgorzata Michalska-Jakubus, Ulf Muller-Ladner, Laura Nuno-Nuno, Rebecca Overbury, Carmen Pizzorni, Mislav Radic, Divya Ramadoss, Angelo Ravelli, Silvia Rosina, Clara Udaondo, Merlijn J. van den Berg, Ariane L. Herrick, Alberto Sulli, Vanessa Smith
Summary: This study aimed to standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). The results showed significant differences in NVC assessment between jRMD subgroups and HCs.
Article
Rehabilitation
Andrea Martina Aegerter, Manja Deforth, Thomas Volken, Venerina Johnston, Hannu Luomajoki, Holger Dressel, Julia Dratva, Markus Josef Ernst, Oliver Distler, Beatrice Brunner, Gisela Sjogaard, Markus Melloh, Achim Elfering
Summary: This study investigated the effect of a multi-component intervention on neck pain-related work productivity loss among Swiss office workers. The results showed that the intervention had a significant reduction in neck pain-related work productivity loss, with implications for employers, employees, and policy makers.
JOURNAL OF OCCUPATIONAL REHABILITATION
(2023)
Article
Nutrition & Dietetics
Roberta De Vito, Federica Fiori, Monica Ferraroni, Silvia Cavalli, Roberto Caporali, Francesca Ingegnoli, Maria Parpinel, Valeria Edefonti
Summary: A recent Italian study found that higher consumption of olive oil and nuts may have a positive effect on disease activity in rheumatoid arthritis (RA), especially for patients with more severe or long-standing forms of the disease. Increasing intake of olive oil, olives, and nuts may be beneficial for improving disease activity in RA.
Article
Health Care Sciences & Services
Chiara Pellicano, Amalia Colalillo, Giuseppina Cusano, Andrea Palladino, Marica Pellegrini, Cinzia Anna Maria Calla, Giorgia Mazzuccato, Valeria Carnazzo, Stefano Pignalosa, Luigi Di Biase, Mariapaola Marino, Umberto Basile, Edoardo Rosato
Summary: This study assessed the serum immunoglobulin G (IgG) subclasses in systemic sclerosis (SSc) patients and their influence on the main complications of the disease. The results showed that SSc patients had lower levels of total IgG, IgG1, and IgG3 compared to healthy controls. Logistic regression analysis found that IgG3 was associated with diffusing capacity of the lung for carbon monoxide (DLco), modified Rodnan skin score (mRSS), and radiological interstitial lung disease (ILD).
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Article
Chemistry, Medicinal
Barbara Ruaro, Murray Baron, Edoardo Rosato, Romeo Martini, Marco Confalonieri
Article
Peripheral Vascular Disease
Chiara Pellicano, Amalia Colalillo, Edoardo Rosato
Summary: This study aimed to evaluate the difference in serum KYNA levels in SSc patients with different stages of microvascular damage. The results showed that SSc patients with late NVC pattern had significantly lower median PDG and serum KYNA compared to those with early and active NVC patterns. Additionally, SSc patients without PDG had significantly lower serum KYNA compared to those with PDG. Conclusion: KYNA may be associated with early endothelial dysfunction in SSc patients.
MICROVASCULAR RESEARCH
(2023)
Article
Rheumatology
Rossella De Angelis, Valeria Riccieri, Edoardo Cipolletta, Nicoletta Del Papa, Francesca Ingegnoli, Silvia Bosello, Amelia Spinella, Greta Pellegrino, Marco de Pinto, Silvia Papa, Giuseppe Armentaro, Dilia Giuggioli
Summary: Nailfold videocapillaroscopy findings were compared in SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH). The results showed that patients with SSc-PAH had more severe abnormalities in nailfold videocapillaroscopy. These findings may be helpful in predicting the diagnosis of PAH.
Article
Nutrition & Dietetics
Roberta De Vito, Maria Parpinel, Michela Carola Speciani, Federica Fiori, Rachele Bianco, Roberto Caporali, Francesca Ingegnoli, Isabella Scotti, Tommaso Schioppo, Tania Ubiali, Maurizio Cutolo, Giuseppe Grosso, Monica Ferraroni, Valeria Edefonti
Summary: Through a recent cross-sectional study in Italy, we found that consuming half a pizza more than once a week had beneficial effects on disease activity in rheumatoid arthritis (RA) patients, with significant reductions in disease severity. Mozzarella cheese and olive oil, among the pizza-related food items, showed particularly beneficial effects in patients with more severe RA. Future cohort studies are needed to confirm these findings.
Review
Medicine, General & Internal
Maria Martin-Lopez, Patricia E. Carreira
Summary: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis. Interstitial lung disease (ILD) is the leading cause of death in patients with SSc. The management of SSc-ILD has shifted to include targeted biologic and antifibrotic therapies.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Health Care Sciences & Services
Chiara Pellicano, Amalia Colalillo, Valeria Carnazzo, Serena Redi, Valerio Basile, Mariapaola Marino, Umberto Basile, Edoardo Rosato
Summary: This study investigated the influence of CD21(low) B cells, IL-4, and IL-21 on joint involvement in SSc patients. The study found that elevated levels of CD21(low) B cells, IL-4, and IL-21 were associated with higher articular disease activity in patients.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Correction
Rheumatology
Karin Melsens, Maurizio Cutolo, Dieneke Schonenberg-Meinem, Ivan Foeldvari, Maria C. Leone, Yora Mostmans, Valerie Badot, Rolando Cimaz, Joke Dehoorne, Ellen Descheppe, Tracy Frech, Johanna Hernandez-Zapata, Francesca Ingegnoli, Archana Khan, Dorota Krasowska, Hartwig Lehmann, Ashima Makol, Miguel A. Mesa-Navas, Malgorzata Michalska-Jakubus, Ulf Mueller-Ladne, Laura Nuno-Nuno, Rebecca Overbury, Carmen Pizzorni, Mislav Radic, Divya Ramadoss, Angelo Ravelli, Silvia Rosina, Clara Udaondo, Merlijn J. van den Berg, Ariane L. Herrick, Alberto Sulli, Vanessa Smith
Article
Rheumatology
Tea Gegenava, Federico Fortuni, Nina Marijn van Leeuwen, Anders H. Tennoe, Anna-Maria Hoffmann-Vold, Ruxandra Jurcut, Adrian Giuca, Laura Groseanu, Felix Tanner, Oliver Distler, Jeroen J. Bax, Jeska De Vries-Bouwstra, Nina Ajmone Marsan
Summary: This study aimed to assess the impact of sex differences on cardiovascular outcomes in patients with systemic sclerosis (SSc). The results showed that male SSc patients had a higher incidence of cardiovascular events, and the adjusted left ventricular global longitudinal strain may explain this gender difference in outcomes.
Letter
Rheumatology
Paola Vidal-Montal, Marion Thomas, Alice Combier, Alexia Steelandt, Corinne Miceli-Richard, Anna Molto, Javier Narvaez, Joan Miquel Nolla, Yannick Allanore, Jerome Avouac