Marie-Pier Tétreault1, Peter Kahrilas2. 1. Department of Medicine, Gastroenterology and Hepatology Division, Northwestern University Feinberg School of Medicine, 15-753 Tarry Building, 300 East Superior Street, Chicago, IL, 60611-3010, USA. marie-pier.tetreault@northwestern.edu. 2. Department of Medicine, Gastroenterology and Hepatology Division, Northwestern University Feinberg School of Medicine, 15-753 Tarry Building, 300 East Superior Street, Chicago, IL, 60611-3010, USA.
Abstract
PURPOSE OF REVIEW: Esophageal dysfunction is common in systemic sclerosis (SSc) patients. Limited treatment options are available for scleroderma esophageal disease. Here, we discuss recent updates on the diagnosis, treatment, and characterization that have been made in patients with scleroderma esophageal disease. RECENT FINDINGS: In the past few years, novel diagnostic tools have provided insight into esophageal dysmotility in SSc patients. New drugs are being tested and might improve symptoms and quality of life in SSc patients with esophageal dysfunction. Molecular stratification methods have facilitated the identification of molecular signatures in the esophagus of SSc patients. The Friend leukemia integration 1 (Fli1) conditional knockout mouse is the first animal model to report an esophageal phenotype with SSc features. The clinical presentation in SSc patients with esophageal dysfunction is heterogeneous, complicating diagnosis and management. The improvement of diagnostic tools for esophageal symptoms and dysfunction and the use of molecular approaches in SSc mouse models and patient biopsies offer an opportunity to improve the characterization of SSc esophageal disease, which should help improve management and treatment decisions.
PURPOSE OF REVIEW: Esophageal dysfunction is common in systemic sclerosis (SSc) patients. Limited treatment options are available for scleroderma esophageal disease. Here, we discuss recent updates on the diagnosis, treatment, and characterization that have been made in patients with scleroderma esophageal disease. RECENT FINDINGS: In the past few years, novel diagnostic tools have provided insight into esophageal dysmotility in SSc patients. New drugs are being tested and might improve symptoms and quality of life in SSc patients with esophageal dysfunction. Molecular stratification methods have facilitated the identification of molecular signatures in the esophagus of SSc patients. The Friend leukemia integration 1 (Fli1) conditional knockout mouse is the first animal model to report an esophageal phenotype with SSc features. The clinical presentation in SSc patients with esophageal dysfunction is heterogeneous, complicating diagnosis and management. The improvement of diagnostic tools for esophageal symptoms and dysfunction and the use of molecular approaches in SSc mouse models and patient biopsies offer an opportunity to improve the characterization of SSc esophageal disease, which should help improve management and treatment decisions.
Authors: Boudewijn F Kessing; Albert J Bredenoord; Pim W Weijenborg; Gerrit J M Hemmink; Clara M Loots; A J P M Smout Journal: Am J Gastroenterol Date: 2011-08-16 Impact factor: 10.864
Authors: Michael D Crowell; Sarah B Umar; W Leroy Griffing; John K DiBaise; Brian E Lacy; Marcelo F Vela Journal: Clin Gastroenterol Hepatol Date: 2016-09-06 Impact factor: 11.382
Authors: Dustin A Carlson; Jacqueline E Prescott; Emma Germond; Darren Brenner; Mary Carns; Chase S Correia; Marie-Pier Tetreault; Zsuzsanna H McMahan; Monique Hinchcliff; Wenjun Kou; Peter J Kahrilas; Harris R Perlman; John E Pandolfino Journal: Neurogastroenterol Motil Date: 2021-10-28 Impact factor: 3.960