Review
Rheumatology
Timothy Kaniecki, Tsion Abdi, Zsuzsanna H. McMahan
Summary: Systemic sclerosis often affects the gastrointestinal tract, leading to impaired function and significant morbidity. This article consolidates current guidelines and proposes a symptom-based framework for diagnosis and management, while also identifying areas in need of further research.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(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
Pharmacology & Pharmacy
Heather Bukiri, Elizabeth R. Volkmann
Summary: Systemic sclerosis is a rare autoimmune disease with unknown cause. The lack of disease modifying therapies has contributed to its high mortality. Recent randomized controlled trials have provided valuable insights into the pathogenesis and potential treatments for systemic sclerosis, leading to the approval of novel therapies for interstitial lung disease associated with systemic sclerosis.
CURRENT OPINION IN PHARMACOLOGY
(2022)
Article
Allergy
Marie Elise Truchetet, Nicolo C. Brembilla, Carlo Chizzolini
Summary: Systemic sclerosis (SSc) is a disease characterized by skin and internal organ fibrosis, vascular modifications, and autoimmunity. It predominantly affects women and has heterogeneous clinical presentations and courses. The pathogenesis involves genetic predisposition, environmental factors, and epigenetic modifications that lead to immune-inflammatory dysregulation and abnormal cell behavior. Myofibroblasts play a crucial role in the disease by promoting fibrosis and matrix deposition. Other cell types, such as pericytes, platelets, and keratinocytes, are also involved. T cell subsets and autoantibodies contribute to fibroblast and endothelial cell dysfunction. Recent studies have revealed heterogeneity in SSc cell differentiation and functional states. Understanding the heterogeneity and pathogenic mechanisms will aid in developing personalized therapeutic approaches.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2023)
Article
Multidisciplinary Sciences
Claire J. J. Han, Kerryn W. Reding, Matthew F. Kalady, Rachel Yung, Heather Greenlee, Electra D. Paskett
Summary: This study investigated the persistent gastrointestinal symptoms experienced by colorectal cancer survivors after cancer treatments. The results showed that 81% of the survivors experienced persistent gastrointestinal symptoms, with bloating/gas being the most prevalent and severe symptom, followed by constipation, diarrhea, and abdominal/pelvic pain. Risk factors for gastrointestinal symptoms included shorter time since diagnosis, advanced cancer stage, high psychological distress, poor dietary habits, and low physical activity.
Review
Oncology
Maria Cecilia Mathias-Machado, Victor Hugo Fonseca de Jesus, Leandro Jonata de Carvalho Oliveira, Marina Neumann, Renata D'Alpino Peixoto
Summary: Gastrointestinal stromal tumors (GISTs) are malignant mesenchymal tumors arising from the intestinal pacemaker cells of Cajal. They comprise a heterogenous group of tumors due to a variety of molecular alterations. The most common gain-of-function mutations in GISTs are either in the KIT (60-70%) or platelet-derived growth factor receptor alpha (PDGFRA) genes (10-15%), which are mutually exclusive.
Review
Medicine, General & Internal
Adan Luquez-Mindiola, Alexis Javier Atuesta, Andres Jose Gomez-Aldana
Summary: Systemic sclerosis is an autoimmune disease characterized by vascular disease, skin fibrosis, and internal organ dysfunction. Gastrointestinal involvement, predominantly affecting the esophagus, small bowel, and rectum, is the most common complication, impacting up to 90% of patients and influencing patients' quality of life and prognosis.
WORLD JOURNAL OF CLINICAL CASES
(2021)
Review
Pharmacology & Pharmacy
Yunjing He, Jingrui Zheng, Binbin Ye, Yongzhao Dai, Ke Nie
Summary: Chemotherapy-induced gastrointestinal toxicity is a major concern for cancer patients, affecting their quality of life and treatment outcomes. The condition involves various factors such as gut microbiota disorders, inflammatory responses, and intestinal neurotoxicity. Traditional Chinese medicine has shown promise as a complementary therapy for managing gastrointestinal toxicity.
BIOCHEMICAL PHARMACOLOGY
(2023)
Article
Rheumatology
Amelie Nicolas, Sylvie Leroy, Luc Mouthon, Yurdagul Uzunhan, Vincent Cottin, Arsene Mekinian, Viviane Queyrel, Eric Hachulla, Benoit Gachet, David Launay, Nihal Martis
Summary: This study provides an overview of the diagnostic approaches, follow-up, and treatment strategies used in France for the management of SSc-associated ILD. The results highlight the heterogeneity and gaps in current clinical practices, calling for improvement and standardization.
THERAPEUTIC ADVANCES IN MUSCULOSKELETAL DISEASE
(2023)
Article
Rheumatology
Audrey D. Nguyen, Kristofer Andreasson, Zsuzsanna H. McMahan, Heather Bukiri, Natalie Howlett, Venu Lagishetty, Sungeun Melanie Lee, Jonathan P. Jacobs, Elizabeth R. Volkmann
Summary: This study evaluated the relationship between gastrointestinal microbiota composition and symptoms in systemic sclerosis (SSc) patients, and compared the symptoms and microbiota composition between SSc patients adhering to a low versus non-low fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAP) diet. The results showed that severe gastrointestinal symptoms were associated with dysbiosis of the microbiota. However, a low FODMAP diet did not significantly affect the microbiota composition or reduce SSc gastrointestinal symptoms.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2023)
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)
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)
Review
Medicine, General & Internal
Djulio Cesar Zanin-Silva, Maynara Santana-Goncalves, Marianna Yumi Kawashima-Vasconcelos, Maria Carolina Oliveira
Summary: Systemic Sclerosis (SSc) is an autoimmune disease characterized by immune dysregulation, tissue fibrosis and vascular dysfunction. Understanding and treating endothelial damage is crucial, with pharmacological and cellular therapy approaches being used to improve vascular pathology.
FRONTIERS IN MEDICINE
(2021)
Article
Immunology
Giuseppe Murdaca, Giovanni Noberasco, Dario Olobardi, Claudio Lunardi, Matteo Maule, Lorenzo Delfino, Massimo Triggiani, Chiara Cardamone, Devis Benfaremo, Gianluca Moroncini, Angelo Vacca, Nicola Susca, Sebastiano Gangemi, Paola Quattrocchi, Laura Sticchi, Giancarlo Icardi, Andrea Orsi
Summary: This paper provides up-to-date evidence on vaccination strategies for patients with systemic sclerosis (SSc) to help prevent and manage infectious diseases, covering a range of vaccines including SARS-CoV-2, seasonal influenza, and others.
Review
Rheumatology
Elizabeth R. Volkmann, Zsuzsanna McMahan
Summary: This article reviews the assessment and treatment strategies for gastrointestinal involvement in patients with systemic sclerosis (SSc) and provides updates on recent research contributions in this area. The findings offer opportunities to enhance patient care and identify future research directions.
CURRENT OPINION IN RHEUMATOLOGY
(2022)