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Sjögren-related cardiomyopathy presenting with cardiogenic shock

Yahia Al Turk1, Alejandro Lemor2,3, Mohamed Fayed4

  • 1Internal Medicine, Henry Ford Health System, Detroit, Michigan, USA yaltur1@hfhs.org.

BMJ Case Reports
|October 20, 2021
PubMed

Insights

This case report highlights Sjögren disease as a rare cause of non-ischaemic cardiomyopathy. Early consideration of autoimmune processes is crucial for diagnosis and management of heart failure in these patients.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Non-ischaemic cardiomyopathy is associated with various autoimmune diseases.
  • Sjögren disease is rarely reported as a cause of cardiomyopathy.

Observation:

  • A 69-year-old woman with Sjögren disease presented with cardiogenic shock.
  • Cardiac MRI showed apical septal late gadolinium enhancement, suggesting an autoimmune etiology.
  • Other causes of cardiomyopathy were excluded.

Findings:

  • The patient's presentation was attributed to underlying Sjögren disease.
  • Treatment with steroids and heart failure therapy was initiated.
  • The patient ultimately died after declining heart transplantation.

Implications:

  • Sjögren disease-related cardiomyopathy lacks established diagnostic criteria and treatment guidelines.
  • Diagnosis requires considering autoimmune processes after excluding other causes.
  • This case underscores the importance of recognizing rare autoimmune etiologies of cardiomyopathy.

Keywords:
Sjogren's syndromecardiovascular medicineheart failureintensive care

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