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Immunoglobulin G4-related coronary periarteritis in a patient presenting with myocardial ischemia

Jun Tanigawa1, Masahiro Daimon, Motonobu Murai

  • 1Department of Cardiology, Osaka Medical College, Osaka 569-8686, Japan.

Human Pathology
|March 10, 2012
PubMed

Insights

Immunoglobulin G4-related disease can affect the heart, as seen in a patient with coronary periarteritis. This condition mimicked acute coronary syndrome, highlighting the need for broader diagnostic considerations.

Area of Science:

  • Cardiovascular Medicine
  • Immunology
  • Rheumatology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition affecting various organs.
  • Emerging evidence suggests potential cardiovascular involvement in IgG4-RD.
  • Accurate diagnosis and management of IgG4-RD cardiovascular manifestations remain challenging.

Observation:

  • A 66-year-old male presented with chest symptoms mimicking acute coronary syndrome.
  • Computed tomography coronary angiography revealed coronary artery stenosis and significant periarterial thickening.
  • Intraoperative findings showed an elastic-hard periarterial mass encasing the left circumflex artery.

Findings:

  • Serum immunoglobulin G4 (IgG4) levels were markedly elevated at 564 mg/dL.
  • Histopathological analysis confirmed lymphoid follicles and IgG4-positive plasma cell infiltration.
  • The diagnosis was established as IgG4-related coronary periarteritis with myocardial ischemia.

Implications:

  • This case underscores the importance of considering IgG4-RD in cardiovascular presentations, especially with elevated IgG4 levels.
  • IgG4-related coronary periarteritis can lead to significant myocardial ischemia, necessitating specific diagnostic and therapeutic approaches.
  • Further research is warranted to elucidate the pathophysiology and long-term cardiovascular outcomes of IgG4-RD.

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