Article
Rheumatology
Jina Yeo, Ju Yeon Kim, Mi Hyeon Kim, Jun Won Park, Jin Kyun Park, Eun Bong Lee
Summary: This study evaluated the clinical utility and reliability of the breath-holding test (BHT) in assessing cardiopulmonary function in patients with SSc. The results showed that BHT had significant correlations with the Borg dyspnea scale, SHAQ, and pulmonary parameters, suggesting that it may be a useful surrogate marker of pulmonary capacity in SSc patients.
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
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
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
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)
Article
Rheumatology
Maya Malaab, Ludivine Renaud, Naoko Takamura, Kip D. Zimmerman, Willian A. da Silveira, Paula S. Ramos, Sandra Haddad, Marc Peters-Golden, Loka R. Penke, Bethany Wolf, Gary Hardiman, Carl D. Langefeld, Thomas A. Medsger, Carol A. Feghali-Bostwick
Summary: Our study identified distinct molecular features in dermal fibroblasts of systemic sclerosis patients, including dysregulated transcription factors and microRNAs, suggesting a role for epigenetic dysregulation in disease susceptibility. This highlights the potential for using epigenetic modifiers as future therapies in systemic sclerosis.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Rheumatology
Francesco Zulian, Gloria Lanzoni, Biagio Castaldi, Alessandra Meneghel, Francesca Tirelli, Elisabetta Zanatta, Giorgia Martini
Summary: This study reports the first case series of patients with ssJSSc, a rare condition in childhood characterized by no skin involvement. Compared to classic JSSc, ssJSSc showed a longer diagnostic delay, higher frequency of cardiac involvement, and worse outcomes.
Review
Gastroenterology & Hepatology
Brigida Barberio, Pierfrancesco Visaggi, Edoardo Savarino, Nicola de Bortoli, Christopher J. Black, Alexander C. Ford
Summary: This study conducted a network meta-analysis to evaluate the efficacy and safety of different medical therapies for endoscopy-negative reflux disease. The results showed that proton pump inhibitors (PPIs) are superior to other drugs in treating this condition. Future research should focus on better patient classification and investigating the efficacy of other drugs.
NEUROGASTROENTEROLOGY AND MOTILITY
(2023)
Review
Gastroenterology & Hepatology
Pierfrancesco Visaggi, Matteo Ghisa, Elisa Marabotto, Arianna Venturini, Delio Stefani Donati, Massimo Bellini, Vincenzo Savarino, Nicola de Bortoli, Edoardo Savarino
Summary: Eosinophilic esophagitis (EoE) is a growing cause of chronic esophageal morbidity. It is characterized by eosinophilic infiltrates of the esophagus and organ dysfunction. Esophageal motor abnormalities may contribute to symptoms and should be considered in treatment and evaluation of patients with EoE.
Editorial Material
Gastroenterology & Hepatology
Pierfrancesco Visaggi, Edoardo Vincenzo Savarino
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2023)
Article
Health Care Sciences & Services
Margherita Zen, Mariele Gatto, Roberto Depascale, Francesca Regola, Micaela Fredi, Laura Andreoli, Franco Franceschini, Maria Letizia Urban, Giacomo Emmi, Fulvia Ceccarelli, Fabrizio Conti, Alessandra Bortoluzzi, Marcello Govoni, Chiara Tani, Marta Mosca, Tania Ubiali, Maria Gerosa, Enrica P. Bozzolo, Valentina Canti, Paolo Cardinaletti, Armando Gabrielli, Giacomo Tanti, Elisa Gremese, Ginevra De Marchi, Salvatore De Vita, Serena Fasano, Francesco Ciccia, Giulia Pazzola, Carlo Salvarani, Simone Negrini, Andrea Di Matteo, Rossella De Angelis, Giovanni Orsolini, Maurizio Rossini, Paola Faggioli, Antonella Laria, Matteo Piga, Alberto Cauli, Salvatore Scarpato, Francesca Wanda Rossi, Amato De Paulis, Enrico Brunetta, Angela Ceribelli, Carlo Selmi, Marcella Prete, Vito Racanelli, Angelo Vacca, Elena Bartoloni, Roberto Gerli, Elisabetta Zanatta, Maddalena Larosa, Francesca Saccon, Andrea Doria, Luca Iaccarino
Summary: This study assessed the efficacy of belimumab in treating joint and skin manifestations in a nationwide cohort of SLE patients. The results showed that belimumab significantly improved joint and skin symptoms and reduced the use of glucocorticoids. A significant proportion of patients who did not achieve complete remission initially achieved remission during follow-up.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Review
Gastroenterology & Hepatology
Pierfrancesco Visaggi, Brigida Barberio, Giulio Del Corso, Nicola de Bortoli, Christopher J. Black, Alexander C. Ford, Edoardo Savarino
Summary: A network meta-analysis was conducted to compare the efficacy of different drugs for active eosinophilic esophagitis (EoE). The results showed that most available drugs were more effective than placebo in treating EoE. However, significant heterogeneity among the trials hindered the establishment of a solid therapeutic hierarchy.
Letter
Cardiac & Cardiovascular Systems
Roberto G. Carbone, Simone Negrini, Francesco Puppo
HEART LUNG AND CIRCULATION
(2023)
Review
Oncology
Alessandro Massano, Luisa Bertin, Fabiana Zingone, Andrea Buda, Pierfrancesco Visaggi, Lorenzo Bertani, Nicola de Bortoli, Matteo Fassan, Marco Scarpa, Cesare Ruffolo, Imerio Angriman, Cristina Bezzio, Valentina Casini, Davide Giuseppe Ribaldone, Edoardo Vincenzo Savarino, Brigida Barberio
Summary: This review summarizes the evidence on the association between inflammatory bowel disease (IBD) and extraintestinal cancers (EICs), as well as the safety and management of immunomodulators and biologics for IBD patients with prior or current EICs.
Review
Pharmacology & Pharmacy
Diego Bagnasco, Edoardo Vincenzo Savarino, Mona-Rita Yacoub, Fulvio Braido, Maria Giulia Candeliere, Edoardo Giannini, Giovanni Passalacqua, Elisa Marabotto
Summary: Type 2 inflammation plays a crucial role in various diseases, and drugs originally developed for severe asthma have shown potential in treating eosinophilic esophagitis. Promising results from clinical trials suggest that these drugs may also be used in gastroenterology in the future.
Article
Pharmacology & Pharmacy
R. G. Carbone, A. Monselise, R. A. Filiberti, D. Penna, S. Negrini, F. Puppo
Summary: The aim of this study was to evaluate the predictive value of New York Heart Association (NYHA) class and systolic pulmonary artery pressure (sPAP) in major interstitial lung diseases (ILD) including idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), hypersensitivity pneumonitis (HP) and other ILD like granulomatosis with polyangiitis (GPA). Analysis of 104 ILD patients showed that NYHA class and sPAP were negatively associated with survival, indicating worse prognosis for IPF and NSIP compared to HP and GPA patients.
EUROPEAN REVIEW FOR MEDICAL AND PHARMACOLOGICAL SCIENCES
(2023)
Article
Respiratory System
Roberto G. Carbone, Giovanni Bottino, Simone Negrini, Francesco Puppo
Summary: Chronic obstructive pulmonary disease (COPD) is a widely diffuse group of diseases that result in airflow blockage and persistent respiratory symptoms. Forced expiratory flow (FEF25-75) is a useful measurement for early COPD diagnosis. We report a case in which a patient showed no response to LAMA treatment but significant improvement with LAMA-LABA combination therapy. This highlights the importance of FEF25-75 evaluation and the efficacy of LAMA-LABA therapy for small airway obstruction.
RESPIRATORY MEDICINE CASE REPORTS
(2023)
Review
Nutrition & Dietetics
Fabiana Zingone, Luisa Bertin, Daria Maniero, Michela Palo, Greta Lorenzon, Brigida Barberio, Carolina Ciacci, Edoardo Vincenzo Savarino
Summary: Most adverse reactions to food are self-reported by patients and not validated by tests. Our study aims to clarify the myths and reality of common food intolerances, providing clinicians with guidance for diagnosis and treatment. The review finds that many food intolerances lack sufficient evidence and lead to unnecessary dietary restrictions for patients.
Article
Nutrition & Dietetics
Gianluca Rizzo, Luciana Baroni, Chiara Bonetto, Pierfrancesco Visaggi, Mattia Orazzini, Irene Solinas, Giada Guidi, Jessica Pugliese, Giulia Scaramuzza, Filippo Ovidi, Irene Buselli, Massimo Bellini, Edoardo V. Savarino, Nicola de Bortoli
Summary: This study investigated the association between diet and GERD, and found that individuals on a vegan diet had a significantly lower risk of developing GERD and reported a worse quality of life.
Article
Gastroenterology & Hepatology
Davide Giuseppe Ribaldone, Giulia Testa, Bram Verstockt, Tamas Molnar, Edoardo Savarino, Carsten Schmidt, Sophie Vieujean, Niels Teich, Corina Meianu, Pascal Juillerat, Nathan Grellier, Triana Lobaton
Summary: There is a lack of data on the effectiveness and safety of Janus kinase inhibitors and sphingosine-1-phosphate receptor modulators in antibiotic refractory chronic pouchitis (CARP). This study retrospectively collected data on JAK inhibitor or S1P receptor modulator treatments for CARP and found that small molecules may be a suitable option for CARP refractory to multiple biologics, but further investigation is warranted.
JOURNAL OF CROHNS & COLITIS
(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)
