IgG4-related disease involving coronary and pulmonary arteries: a case report and literature review.

ABSTRACT

Background: IgG4-related disease (IgG4-RD) is an inflammation-mediated autoimmune disease characterized by infiltration of IgG4 plasma cells in target organs, storiform fibrosis and obliterative phlebitis, accompanied by or without elevated serum IgG4 concentrations. Multiple sites can be involved, including large vessels. Coronary and pulmonary arteries are less involved, while simultaneous involvement of coronary and pulmonary arteries is less reported. This case is unique in terms of simultaneous involvement of coronary and pulmonary arteries in a female patient with possible IgG4-RD and the first review of relevant domestic literature. Case Description This case is a middle-aged female patient with both coronary artery and pulmonary artery involvement, with cardiac insufficiency as the main manifestation. Cardiac ultrasound revealed the cardiac insufficiency and abnormal wrapping of multiple arteries. Imaging examinations including coronary artery computed tomography angiography (CTA), pulmonary artery CTA and cardiac magnetic resonance imaging (MRI) further confirmed the lesions of the left main coronary artery, anterior descending branch, circumflex branch and pulmonary artery. Then the patient was diagnosed with possible IgG4-RD. After glucocorticoid treatment, the patient's clinical symptoms and cardiac function improved, and her serum IgG4 levels decreased. Conclusions: When the arterial system is involved in IgG4 disease, it is known as IgG4-related artery disease. Combined with the case of this patient, this paper reviewed the literature on IgG4-related artery disease, and searched and summarized the related domestic literature on coronary/pulmonary artery disease to improve people's understanding of IgG4-related artery disease.