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Severe triple vessel disease secondary to IgG4-related coronary periarteritis

Guillaume Goudot1, Mounica Yanamandala1, Richard Mitchell2

  • 1Department of Medicine, Cardiovascular Division, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Journal of Cardiac Surgery
|November 15, 2022
PubMed

Insights

Immunoglobulin G4-related disease (IgG4-RD) can cause vascular issues. This case shows IgG4-RD affecting coronary arteries, leading to improved angina after treatment with prednisone and rituximab.

Area of Science:

  • Cardiovascular Medicine
  • Rheumatology
  • Immunology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a rare, chronic, fibroinflammatory condition.
  • Vascular manifestations, including periarteritis, are recognized complications of IgG4-RD.
  • Early diagnosis and treatment are crucial for managing IgG4-RD and preventing organ damage.

Observation:

  • A 41-year-old male presented with stress-induced angina.
  • Coronary angiography revealed severe triple-vessel coronary artery disease.
  • Surgical findings indicated significant perivascular thickening around the coronary arteries and aorta.

Findings:

  • Pathological examination of the aorta confirmed IgG4-related disease.
  • The patient's stress angina symptoms significantly improved post-operatively.
  • Treatment with prednisone and rituximab was initiated following diagnosis.

Implications:

  • This case highlights the importance of considering IgG4-RD in patients with unexplained vascular inflammation.
  • Aggressive immunosuppressive therapy, including corticosteroids and B-cell depletion, can effectively manage IgG4-RD-associated cardiovascular complications.
  • Further research is warranted to elucidate the precise mechanisms of IgG4-RD in vascular disease and optimize treatment strategies.
Abstract

Keywords:
IgG4-related diseasecoronary artery diseasecoronary vasculitis

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