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IgG4-Related Aortitis as a Driver of Coronary Artery Stenosis and Ischemic Cardiomyopathy

Nicholas Olivieri1, Feras Aboukhater1, Nicholas Poellinger2

  • 1Stritch School of Medicine, Loyola University Chicago, Maywood, Illinois, USA.

JACC. Case Reports
|July 18, 2025
PubMed

Insights

IgG4-related disease can cause aortitis, leading to early coronary artery stenosis and ischemic cardiomyopathy. Prompt diagnosis and management are crucial for patients presenting with these conditions.

Area of Science:

  • Cardiology
  • Immunology
  • Pathology

Background:

  • Vasculitis and aortitis are known contributors to early coronary artery stenosis.
  • Ischemic cardiomyopathy can result from coronary artery stenosis.

Observation:

  • A 45-year-old woman with new heart failure and no cardiac risk factors presented with multivessel coronary artery disease.
  • Angiography revealed severe aortic inflammation and thickening, with stenosed coronaries lacking atherosclerosis.
  • Histologic examination confirmed aortitis secondary to IgG4-related disease.

Findings:

  • IgG4-related disease is a recognized cause of aortitis with potential coronary artery involvement.
  • Aortitis can lead to coronary stenosis through vasculitis extension or external compression.

Implications:

  • IgG4-related disease should be considered in cases of unexplained aortitis and early coronary artery disease.
  • Histologic evaluation is often necessary for diagnosing and managing IgG4-related aortitis.
  • Understanding this association is key for effective patient management and treatment strategies.
Abstract

Keywords:
autoimmunecardiovascularmyocardial ischemiamyocardial revascularizationvascular disease

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