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World J Clin Cases. Jul 16, 2021; 9(20): 5408-5419
Published online Jul 16, 2021. doi: 10.12998/wjcc.v9.i20.5408
Esophageal manifestation in patients with scleroderma
Theodoros A Voulgaris, Georgios P Karamanolis
Theodoros A Voulgaris, Georgios P Karamanolis, Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
Author contributions: Voulgaris TA wrote the article; Karamanolis GP was responsible for conception and design of the article, the drafting of the article, making critical revisions and final approval of the article.
Conflict-of-interest statement: There is no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Georgios P Karamanolis, MD, PhD, Assistant Professor, Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Michalakopoulou 176, Athens 11527, Greece. georgekaramanolis@yahoo.co.uk
Received: February 28, 2021
Peer-review started: February 28, 2021
First decision: April 18, 2021
Revised: April 22, 2021
Accepted: May 10, 2021
Article in press: May 10, 2021
Published online: July 16, 2021
Processing time: 128 Days and 14 Hours
Abstract

The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis (SSc). Esophageal involvement may lead to a significant reduction in patient quality of life. The exact pathophysiology is complex and not yet fully elucidated. Ultimately, esophageal smooth muscle becomes atrophied and replaced by fibrous tissue leading to severe motility disturbance of the distal esophagus. Symptoms are mainly attributed to gastroesophageal reflux disease and to esophageal dysmotility. Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease. No formed guidelines exist about the diagnostic modalities used to assess esophageal disease in patients with SSc, though upper gastrointestinal endoscopy is the first and most important modality used as it can reveal alterations commonly observed in patients with SSc. Further exploration can be made by high resolution manometry and pH-impedance study. Proton pump inhibitors remain the mainstay of treatment, while prokinetic agents are commonly used as add-on therapy in patients with symptoms attributed to gastroesophageal reflux disease not responding to standard therapy as well as to motility disturbances. Gastroesophageal reflux disease symptoms in patients with SSc are frequently difficult to manage, and new therapeutic modalities are emerging. The role of surgical treatment is restricted and should only be preserved for resistant cases.

Keywords: Systemic sclerosis; Esophagus; Gastroesophageal reflux disease; Esophageal dysmotility; Proton pump inhibitors

Core Tip: Gastrointestinal manifestations of systemic sclerosis, a rare autoimmune disease, are the most commonly encountered complications of the disease affecting nearly 90% of the systemic sclerosis population. Among the gastrointestinal tract, the esophagus is the most commonly affected. In this review, we will present the current understanding of the pathophysiologic mechanisms of systemic sclerosis, the clinical presentation and diagnosis of esophageal involvement. Finally, we highlight the latest developments in the management of this disease.