Perspectives on the interlinked nature of systemic sclerosis and reflux disease

ABSTRACT

Introduction: Systemic sclerosis (SSc) is a multisystem connective tissue disease, characterized by chronic inflammation and vascular changes that result in esophageal smooth muscle atrophy and fibrosis. Subsequent progressive loss of peristalsis in the distal esophagus and loss of lower esophageal sphincter function lead to problems with the protective barrier and exposure of sensitive tissues to the gastroduodenal contents, a disorder called reflux disease.

Areas covered: Depending on the range, nature and symptoms of the disease, the term ‘reflux disease’ may refer to gastroesophageal reflux, laryngopharyngeal reflux, microaspiration into the airways and silent reflux. Despite the links between these visceral complications, this connection remains controversial. This is due to a lack of complete understanding, the asymptomatic nature of the disease and the limited diagnostic accuracy of tests, which can delay diagnosis. Such delays are problematic, given that the early detection of GERD in SSc patients, the timing of assessment, the treatment of the organs involved are critical aspects of patient prognosis and disease outcome.

Expert commentary: This review summarizes the most recent knowledge about the pathophysiology, diagnosis and prospective treatment of GERD in SSc patients and highlights how innovative technologies applied through an integrative, interdisciplinary approach may soon lead to effective treatment strategies.

KEYWORDS:

• Barrett’s esophagus
• GERD
• interstitial lung disease
• laryngopharyngeal reflux
• microaspiration
• silent reflux
• systemic sclerosis

Article highlights

• The asymptomatic nature of GERD in most early diagnosed SSc patients alarmingly delays effective treatment and is associated with a poor quality of life and a possibly increased risk of esophageal or laryngeal cancer.

• The high prevalence of reflux disease in SSc indicates a probable link between microaspiration and complications in the respiratory tract.

• Innovative technologies, e.g. extremely accurate devices for the diagnosis of reflux, MSA and human intravenous immunoglobulin, are eagerly awaited to track and treat early GERD in SSc.

This box summarizes key points contained in the article.

Acknowledgments

We are grateful to Dr. Parakrama Chandrasoma (Keck School of Medicine, University of Southern California, Los Angeles, CA, USA), Dr. Tom R. DeMeester (Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA) and Dr. Jeffrey H. Peters (University Hospitals Cleveland, Cleveland, OH, USA) for sharing the photos shown in Figure 1(b) [13, 16].

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.