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GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis

Current Rheumatology Reports volume 21, Article number: 42 (2019) Cite this article

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.

Summary

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.

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Fig. 1
Fig. 2

Abbreviations

Fli1:

Friend leukemia integration 1

K14:

Keratin-14

SSc:

Systemic sclerosis

ILD:

Interstitial lung disease

CT:

Computed tomography

PPI:

Proton-pump inhibitor

BI:

Baseline impedance

NERD:

Non-erosive reflux disease

ACPA:

Anti-citrullinated peptide antibodies

RA:

Rheumatoid arthritis

LES:

Lower esophageal sphincter

EGJ:

Esophagogastric junction

IQR:

Interquartile range

HRM:

High-resolution manometry

MRS:

Multiple rapid swallows

EPT:

Esophageal pressure topography

GISSI:

Gastrointestinal Symptoms Severity Index

GIT:

University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract

HRIM:

High-resolution impedance manometry

FLIP:

Functional luminal imaging probe

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Funding

This work was supported by NIH NIDDK P01 DK117824.

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Affiliations

  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 Tétreault & Peter Kahrilas

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P.K. and M.-P.T. wrote the manuscript.

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Correspondence to Marie-Pier Tétreault.

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Tétreault, MP., Kahrilas, P. GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis. Curr Rheumatol Rep 21, 42 (2019). https://doi.org/10.1007/s11926-019-0841-x

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  • DOI: https://doi.org/10.1007/s11926-019-0841-x

Keywords

  • Systemic sclerosis
  • Esophagus
  • Manometry
  • Impedance
  • Esophageal reflux monitoring
  • Functional luminal imaging probe