Esophageal Dysfunction in Systemic Sclerosis: An Update

Abstract

Esophageal motility disorders are prevalent in 90% of patients with systemic sclerosis [scleroderma (SSc)], with an increased mortality rate in patients with severe esophageal involvement. Esophageal smooth muscle damage caused by ischemia, nerve damage, and inflammatory factors may be responsible for discomfort and various complications in these patients. The clinical manifestations are diverse. Most hospitals still use traditional esophageal manometry and 24-h pH monitoring to diagnose esophageal function in patients with SSc. The aim of this review article is to provide an overview of SSc-related esophageal motility disorders and related research progress, including the pathogenesis and clinical features of these disorders and the progress made in endoscopic diagnosis. We also discuss the possible pathogenesis and potential therapeutic targets.

Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis

“…Esophageal involvement manifests as dysphagia, acid reflux, heartburn and retrosternal pain ( 107 ). Long-term esophageal motility disorders in patients with SSc may lead to complications, such as gastroesophageal reflux, esophagitis, esophageal erosion, stricture, ulcers, diverticulum, leukoplakia, Barrett’s esophagus and adenocarcinoma ( 107 ).…”

“…Esophageal involvement manifests as dysphagia, acid reflux, heartburn and retrosternal pain ( 107 ). Long-term esophageal motility disorders in patients with SSc may lead to complications, such as gastroesophageal reflux, esophagitis, esophageal erosion, stricture, ulcers, diverticulum, leukoplakia, Barrett’s esophagus and adenocarcinoma ( 107 ). Although the causal relationship between gastroesophageal reflux and ILD remains unclear, early treatment of esophageal disease could reduce the severity of lung involvement ( 108 ).…”

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

“…In the beginning, vasculopathy may generate irreversible muscle and nerve lesions. Similar to Raynaud’s phenomenon, the vasospasm decreases the muscle blood supply, leading to motor impairment, and platelet and endothelin activation as a response to ischemia [ 60 , 61 ]. As a consequence, there is a disproportionate extracellular matrix formation and aberrant collagen deposition [ 55 , 61 ].…”

A Response to: Letter to the Editor Regarding [Esophageal Dysfunction and Systemic Sclerosis: Drugs Should be Kept in Mind]

“…In our previously published systematic review of esophageal dysfunction in patients with SSc, we summarized the treatment options for esophageal dysfunction in patients with SSc [ 1 ]. Our analysis suggests a general strategy for this group of patients similar to patients with gastroesophageal reflux disease (GERD), and by avoiding the consumption of tobacco, alcohol, and non-steroidal anti-inflammatory drugs.…”