• Kensuke Namba, Daiki Sakai, Hiroshi Mikamo, Yuta Sugizaki, Yoshiya Sugiura, Nobuyuki Hiruta, Yasuo Matsuzawa, and Kaichi Kaneko.
• Division of Rheumatology, Department of Internal Medicine, Toho University Sakura Medical Center, Sakura, Japan.
• Medicine (Baltimore). 2023 Aug 4; 102 (31): e34533e34533.

RationaleAlthough IgG4-related disease (IgG4-RD) can affect various organs, its association with a cardiac mass is exceptionally rare. Here, we report a case of a woman with IgG4-RD and a cardiac mass and discuss 10 similar cases reported previously.Patient ConcernsA 65-year-old woman was referred to our hospital for chest discomfort and back pain.DiagnosesIn accordance with the 2019 ACR/EULAR diagnostic criteria for IgG4-RD, she was diagnosed with IgG4-RD based on dense lymphocytic infiltration on histopathology, IgG/IgG4-positive cell ratio <40%, >10/hpf IgG4-positive cells on immunostaining, and paraspinal zone soft tissue lesions in the chest.InterventionsAn external pacemaker was implanted for the complete atrioventricular block on the electrocardiogram. After the diagnosis of IgG4-RD, she was treated with glucocorticoids and rituximab.OutcomesShe remains under observation without disease recurrence.LessonsIgG4-RD are usually treated with glucocorticoids; however, in cases of a cardiac mass, life-threatening complications may occur and surgery is often needed. Combination therapy with glucocorticoids and rituximab may be effective even in patients with IgG4-RD and cardiac mass, which may avoid the need of invasive treatments, such as surgery.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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