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Related Experiment Videos

Esophageal dysfunction in primary biliary cirrhosis.

A Parés1, L Grande, J Bruix

  • 1Liver Unit, Hospital Clinic i Provincial, Faculty of Medicine, University of Barcelona, Spain.

Journal of Hepatology
|December 1, 1988
PubMed
Summary
This summary is machine-generated.

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Esophageal manometry in primary biliary cirrhosis patients reveals motility dysfunction in those with scleroderma and Sjögren's syndrome. These findings suggest a link between Sjögren's syndrome and esophageal motor disturbances in PBC.

Area of Science:

  • Gastroenterology
  • Autoimmune Diseases
  • Clinical Physiology

Background:

  • Primary biliary cirrhosis (PBC) can be associated with autoimmune conditions like scleroderma and Sjögren's syndrome.
  • Esophageal involvement is a known complication in scleroderma, but its prevalence in PBC patients with or without associated autoimmune diseases requires further investigation.

Purpose of the Study:

  • To investigate the prevalence and characteristics of esophageal motility dysfunction in patients with primary biliary cirrhosis (PBC).
  • To determine if esophageal involvement in PBC is associated with the presence of scleroderma or Sjögren's syndrome.

Main Methods:

  • Esophageal manometry was performed on 18 patients with PBC and 18 age- and sex-matched controls.
  • Patients were assessed for clinical manifestations of scleroderma and screened for Sjögren's syndrome.

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  • Lower esophageal sphincter pressure (LESP) and distal mean wave pressure (DMWP) were measured.

    • Main Results:

    • Three out of four patients with scleroderma (all also had Sjögren's syndrome) exhibited aperistalsis and diminished lower sphincter pressure.
    • Nine patients with Sjögren's syndrome but without scleroderma also showed esophageal manometric disturbances.
    • LESP and DMWP were significantly reduced in patients with scleroderma and those with Sjögren's syndrome compared to controls. Patients without Sjögren's syndrome had normal values.

    Conclusions:

    • Esophageal motility dysfunction is common in primary biliary cirrhosis patients, particularly those with co-existing scleroderma or Sjögren's syndrome.
    • The findings suggest that Sjögren's syndrome may contribute to esophageal motor disturbances in PBC patients.
    • Esophageal manometry is a valuable tool for assessing esophageal involvement in PBC patients with autoimmune comorbidities.