Coronary periarteritis in IgG4-RD: A case series.

Objectives: IgG4-related disease (IgG4-RD) can affect multiple organ systems, with coronary artery involvement being rare. Coronary periarteritis may lead to complications such as myocardial infarction and ischemic cardiomyopathy. This case series characterizes the clinical and radiological features, complications, and treatment strategies in patients with IgG4-RD-associated coronary periarteritis.

Methods: A retrospective review of the Mayo Clinic electronic health record identified 146 patients, of whom 9 met the inclusion criteria for IgG4-RD with coronary periarteritis. Clinical, laboratory, imaging, treatment, and outcome data were analyzed.

Results: The cohort included 9 male patients (mean age: 55.5 ± 5.7 years). The median serum IgG-4 level was 282 mg/dL (interquartile range [IQR], 148-393), while the median C-reactive protein value was 10.8 mg/dL (IQR 7.5-53.6). The mean time from IgG4-RD diagnosis to coronary involvement was 2.5 years. CT angiography detected vessel wall thickening, affecting the right coronary artery (RCA) in five cases, the left anterior descending artery (LAD) in four, and the left main coronary artery in one. Aneurysm formation occurred in two cases. All patients received glucocorticoids, and seven were treated with rituximab. Over a mean follow-up of 7.5 years, one patient developed ischemic heart disease, and another progressed to heart failure. Repeat imaging showed partial or complete resolution of coronary thickening in seven patients.

Conclusion: Coronary artery involvement in IgG4-RD is rare but may lead to aneurysm formation and ischemic complications. CT and MRI are valuable diagnostic tools, and rituximab with glucocorticoids appears effective, though further studies are needed. Key Points • IgG4-related disease can involve the coronary arteries, leading to periarteritis and potential complications such as myocardial infarction and aneurysm formation, highlighting the need for early recognition. • Glucocorticoids and rituximab appear to be effective in managing IgG4-RD-associated coronary periarteritis, but further studies are needed to evaluate long-term outcomes.