Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disorder characterized by elevated serum IgG4 levels and the enlargement and fibrosis of organs. As a rare manifestation, coronary arteries can be affected by IgG4-RD as coronary periarteritis, leading to serious complications such as stenosis or aneurysm. Although coronary periarteritis poses a life-threatening condition, optimal treatment strategies remain unclear due to its extreme rarity. While glucocorticoids have shown efficacy in several reported cases of IgG4-related coronary periarteritis, many cases experience relapse during glucocorticoid tapering. Furthermore, long-term use of glucocorticoids promotes atherosclerosis and increases the risk of major adverse cardiovascular events. Given that rituximab has been reported to be effective in treating IgG4-RD, it may be a potential treatment option for this condition. We present a case of IgG4-related coronary periarteritis, in which the patient achieved and maintained remission with rituximab. Furthermore, our review of the literature identified 17 cases of IgG4-related coronary periarteritis, all of which were successfully treated with rituximab. These findings suggest that rituximab serves as a viable option for both induction and maintenance therapy in IgG4-related coronary periarteritis.
