Clinical Characteristics and Multimodal Imaging Insights of Coronary Involvement in Immunoglobulin G4–Related Disease

Yaqi Du¹Yun Bai¹Yimo Zhou¹Shuang Ding¹Xinrui Wang¹Yibin Xie²Hsin-jung Yang²Debiao Li²Zhaoyang Fan³Guoguang Fan¹Zhe Lou^1*Jiayi Wei^1*Guan Wang^1*

• ¹The First Hospital of China Medical University, Shen Yang, China
• ²Cedars-Sinai Medical Center, Los Angeles, United States
• ³University of Southern California, Los Angeles, United States

Introduction: Coronary involvement in immunoglobulin G4–related disease (IgG4-RD) has remained underexplored despite its risk posed in terms of major adverse cardiovascular events (MACEs). The study provides a comprehensive review, particularly focusing on multimodal imaging characteristics and clinical applicability. Methods: A systematic review was conducted on IgG4-related coronary involvement, supplemented by serial cases from our center included. We analyzed clinical features and multimodal imaging, focusing on the presence or absence of cardiovascular symptoms. Results:A total of 134 IgG4-RD patients with coronary involvement were included and analyzed, including 118 from the literature and 16 from our center. Seven (5%) patients died from secondary myocardial ischemia/infarction. Coronary anomalies commonly affected the left anterior descending artery (LAD) (79%) and presented as diffuse wall thickening or periarterial soft tissue encasement (85%). Stenosis was frequent (47%) and often secondary. Symptoms, primarily induced by myocardial ischemia or infarction (84%), were largely due to stenosis (68%). Chest computed tomography (CT) and coronary computed tomography angiography (CTA) were the primary imaging modalities (81%), particularly in symptomatic cases (88%). Positron emission tomography-computed tomography (PET-CT) was applied in 55 patients (41%) and often in asymptomatic cases (51%). CMR, though less adopted (23%), demonstrated potential in detecting coronary lesions (77%). Glucocorticoid therapy is the most common (76%), with the best response of periarterial encasement (66%). Surgery was less common (32%), primarily being applied to aneurysms (63%). Conclusion: Coronary involvement in IgG4-RD presents four phenotypes, sometimes with an insidious onset and as the sole affected site, poses a potential risk for MACEs. Multimodal imaging is essential for early diagnosis and effective monitoring, with coronary CMR showing promise for early detection without the risk of radiation-induced inflammation and fibrosis.