Coronary periarteritis in IgG4-RD: A 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.