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Coronary periarteritis in IgG4-RD: A case series

Georges El Hasbani1, Daniel Larson2, Matthew J Koster3

  • 1Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55906, USA. ElHasbani.georges@mayo.edu.

Clinical Rheumatology
|September 10, 2025
PubMed

Insights

IgG4-related disease (IgG4-RD) can rarely affect coronary arteries, causing periarteritis and potential heart complications. Treatment with glucocorticoids and rituximab showed effectiveness in this case series.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibroinflammatory condition.
  • Coronary artery involvement in IgG4-RD, presenting as periarteritis, is uncommon.
  • Coronary periarteritis can lead to serious cardiac complications, including myocardial infarction and ischemic cardiomyopathy.

Purpose of the Study:

  • To characterize the clinical and radiological features of IgG4-RD-associated coronary periarteritis.
  • To identify complications associated with this rare condition.
  • To evaluate treatment strategies and outcomes in affected patients.

Main Methods:

  • Retrospective review of electronic health records at Mayo Clinic.
  • Inclusion criteria: diagnosis of IgG4-RD with confirmed coronary periarteritis.
  • Analysis of clinical, laboratory, imaging, treatment, and outcome data for 9 patients.

Main Results:

  • The cohort comprised 9 male patients with a mean age of 55.5 years.
  • Median serum IgG-4 level was 282 mg/dL; median C-reactive protein was 10.8 mg/dL.
  • CT angiography revealed coronary artery wall thickening in most cases, with two instances of aneurysm formation. Glucocorticoids and rituximab were administered. Over 7.5 years of follow-up, one patient developed ischemic heart disease and another heart failure. Seven patients showed resolution of coronary thickening.

Conclusions:

  • IgG4-RD coronary periarteritis is rare but can result in aneurysms and ischemic complications.
  • CT and MRI are valuable for diagnosis.
  • Combined glucocorticoid and rituximab therapy appears effective, warranting further investigation for long-term outcomes.
Abstract

Keywords:
Coronary involvementIgG4-related diseaseRadiologyRituximab

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