Gastrointestinal Manifestations of Systemic Sclerosis
Isabel M. McFarlane,Manjeet S Bhamra,Alexandra Kreps,Sadat Iqbal,Firas Al-Ani,Carla Saladini-Aponte,Christon Grant,Soberjot Singh,Khalid Awwal,Kristaq Koci,Yair Saperstein,Fray Martin M. Arroyo-Mercado,Derek B Laskar,Purna Atluri +13 more
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TLDR
The current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways are presented.Abstract:
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative alterations of the microvasculature leading to fibrosis and loss of function of the skin and internal organs. Gastrointestinal manifestations of SSc are the most commonly encountered complications of the disease affecting nearly 90% of the SSc population. Among these complications, the esophagus and the anorectum are the most commonly affected. However, this devastating disorder does not spare any part of the gastrointestinal tract (GIT), and includes the oral cavity, esophagus, stomach, small and large bowels as well as the liver and pancreas. In this review, we present the current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways. We also discuss the clinical presentation and diagnosis of each part of the GIT affected by SSc. Finally, we highlight the latest developments in the management of this disease, addressing the severe malnutrition that affects this vulnerable patient population and ways to assess and improve the nutritional status of the patients.read more
Citations
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Journal ArticleDOI
Systemic sclerosis - multidisciplinary disease: clinical features and treatment.
Piotr Sobolewski,Maria Maślińska,Marta Wieczorek,Zuzanna Łagun,Aleksandra Malewska,Marek Roszkiewicz,Raman Nitskovich,Elżbieta Szymańska,Irena Walecka +8 more
TL;DR: Different clinical features of systemic sclerosis and the profile of autoantibodies are summarized and recent rules and future perspectives in disease management are discussed.
Journal ArticleDOI
Current management of the gastrointestinal complications of systemic sclerosis.
TL;DR: The most commonly occurring clinical scenarios of gastrointestinal involvement in patients with systemic sclerosis as they present to the gastroenterologist are described, with recommendations for the suggested assessment protocol and therapy in each situation.
Journal ArticleDOI
Ongoing clinical trials and treatment options for patients with systemic sclerosis–associated interstitial lung disease
Dinesh Khanna,Donald P. Tashkin,Christopher P. Denton,Martin W. Lubell,Cristina Vazquez-Mateo,Stephen Wax +5 more
TL;DR: Newer agents targeting the pathogenic mechanisms underlying SSc-ILD, including fibrotic and inflammatory pathways, lymphocytes, cell–cell and cell–extracellular membrane interactions, hold promise for better treatment outcomes, including improved lung function, patient-related outcomes and quality of life.
Journal ArticleDOI
Intestinal Involvement in Systemic Sclerosis: A Clinical Review.
Lazaros I. Sakkas,Theodora Simopoulou,Dimitrios Daoussis,Stamatis-Nick C. Liossis,Spyros P. Potamianos +4 more
TL;DR: Multidisciplinary approach of intestinal manifestations in SSc by gastroenterologists and rheumatologists is required for optimum management.
Journal ArticleDOI
Evaluation and management of esophageal manifestations in systemic sclerosis.
TL;DR: Buspirone, a 5-hydroxytryptamine 1A receptor agonist, could be a putative therapeutic option, as it was found to exert a significant beneficial effect in SSc patients with esophageal involvement, including emerging therapeutic modalities.
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