Background/Purpose: IgG4-related disease (IgG4-RD) is characterized by chronic inflammation and fibrosis, which can affect multiple organs, especially the pancreas, salivary glands, and retroperitoneum (1). It may lead to periaortitis, affecting up to 20% of patients, and in rare cases, coronary periarteritis, which can cause severe complications like myocardial infarction (2). This case series aims to explore the clinical and radiological features of coronary periarteritis in IgG4-RD and discuss complications and treatment options. 1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-51.2. Katz G, Hedgire SH, Stone JR, Perez-Espina S, Fernandes A, Perugino CA, et al. IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement. Semin Arthritis Rheum. 2023;60:152184.

Methods: A review of the electronic health records was conducted using keywords related to IgG4-RD and coronary involvement. Out of 146 patients reviewed, 9 patients had confirmed IgG4-RD with coronary involvement. Data extracted included diagnosis date, serum IgG4 and C-reactive protein levels, imaging findings, cardiac complications, and treatment details.

Results: All 9 patients were male, with a mean age of 55.5 ± 5.7 years at diagnosis. The mean serum IgG-4 level was 738 ± 1280 mg/dL, while the mean C-reactive protein value was 39.9 ± 54.8 mg/L. The average time between diagnosis of IgG4-RD and coronary involvement was 2 years and 6 months. A tissue biopsy showed IgG4-positive plasma cells in all cases. Coronary artery involvement was primarily detected through CT angiography, showing thickening of vessel walls (Table) . The right coronary artery (RCA) was affected in 5 patients, the left anterior descending artery (LAD) in 4, and the left main coronary artery in 1. In 5 patients, the periarteritis involved the entire length of the coronary artery, and 1 patient had an aneurysm of the LAD. Regarding treatment, all patients received glucocorticoids, and 7 patients were additionally treated with rituximab (RTX). Two patients received RTX for induction only, while 4 patients had induction and maintenance RTX infusions. One patient developed ischemic heart disease, and one progressed to heart failure. Follow-up imaging showed partial or complete resolution of vessel thickening in 7 patients.

Conclusion: Coronary artery involvement in IgG4-RD is rare but significant, leading to periarteritis, and subsequently coronary aneurysm formation. The RCA and LAD appear to be most affected in this condition, with imaging techniques such as cardiac CT being effectively used as diagnostic tool. Rituximab has shown promising data in managing coronary involvement, though its use in this context remains limited, ultimately necessitating larger studies.

Patient demographics and clinical features.

CT cardiac demonstrating thickening of the wall of the right coronary artery. H&E, pelvic lymph node biopsy with plasmacytosis. IgG shows most plasma cells express IgG. IgG4 shows 30-40% of plasma cells are IgG4-postive, with over 100/hpf.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/coronary-periarteritis-in-igg4-related-disease/