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Abstract：
BACKGROUND: The heart can be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to summarize the clinical features and efficacy of treatment for IgG4-RD patients with heart involvement.
METHODS: We conducted a retrospective study enrolling 42 IgG4-RD patients with heart involvement from the IgG4-RD cohorts of the Peking Union Medical College Hospital and Beijing An Zhen Hospital, from 2010 to 2022. Clinical, laboratory, radiological data were collected, and treatment responses to glucocorticoids and immunosuppressants were analyzed.
RESULTS: IgG4-related cardiac involvement is a rare part of the IgG4-RD spectrum. The incidences of coronary periarteritis and pericarditis were 1.2%(13/1075) and 3.1%(33/1075), respectively in our cohort. Valvular disease possibly related to IgG4-RD was detected in two patients. None of the patients with myocardial involvement were identified. The average age was 58.2 ± 12.8 years, with a male predominance (76.7%). Coronary artery CT revealed that mass-like and diffuse wall-thickening lesions were the most frequently observed type of coronary periarteritis. Pericarditis presented as pericardial effusion, localized thickening, calcification and mass. After treatment with glucocorticoid and immunosuppressants, all patients achieved a reduced IgG4-RD responder index score and achieved radiological remission. Two patients with coronary peri-arteritis experienced clinical relapses during the maintenance period.
CONCLUSIONS: Cardiac involvement in IgG4-RD is rare and easily overlooked since many patients are asymptomatic, and the diagnosis relies on imaging. Patients showed a satisfactory response to glucocorticoid based treatment.
METHODS: We conducted a retrospective study enrolling 42 IgG4-RD patients with heart involvement from the IgG4-RD cohorts of the Peking Union Medical College Hospital and Beijing An Zhen Hospital, from 2010 to 2022. Clinical, laboratory, radiological data were collected, and treatment responses to glucocorticoids and immunosuppressants were analyzed.
RESULTS: IgG4-related cardiac involvement is a rare part of the IgG4-RD spectrum. The incidences of coronary periarteritis and pericarditis were 1.2%(13/1075) and 3.1%(33/1075), respectively in our cohort. Valvular disease possibly related to IgG4-RD was detected in two patients. None of the patients with myocardial involvement were identified. The average age was 58.2 ± 12.8 years, with a male predominance (76.7%). Coronary artery CT revealed that mass-like and diffuse wall-thickening lesions were the most frequently observed type of coronary periarteritis. Pericarditis presented as pericardial effusion, localized thickening, calcification and mass. After treatment with glucocorticoid and immunosuppressants, all patients achieved a reduced IgG4-RD responder index score and achieved radiological remission. Two patients with coronary peri-arteritis experienced clinical relapses during the maintenance period.
CONCLUSIONS: Cardiac involvement in IgG4-RD is rare and easily overlooked since many patients are asymptomatic, and the diagnosis relies on imaging. Patients showed a satisfactory response to glucocorticoid based treatment.
摘要：
背景：心脏可能与免疫球蛋白（Ig）-G4相关疾病（IgG4-RD）有关。本研究旨在总结IgG4-RD合并心脏受累患者的临床特点及治疗效果。
方法：我们进行了一项回顾性研究，从北京协和医院和北京安贞医院的IgG4-RD队列中纳入了42例IgG4-RD患者，从2010年到2022年。临床,实验室,收集放射学数据，并分析了对糖皮质激素和免疫抑制剂的治疗反应。
结果：与IgG4相关的心脏受累是IgG4-RD谱的罕见部分。冠状动脉周围炎和心包炎的发生率分别为1.2%(13/1075)和3.1%(33/1075),分别在我们的队列中。在两名患者中检测到可能与IgG4-RD相关的瓣膜疾病。没有发现心肌受累的患者。平均年龄58.2±12.8岁,男性占主导地位（76.7%）。冠状动脉CT显示，肿块样和弥漫性壁增厚病变是最常见的冠状动脉周炎类型。心包炎表现为心包积液，局部增厚,钙化和肿块。糖皮质激素和免疫抑制剂治疗后，所有患者的IgG4-RD反应者指数评分均降低,并获得放射学缓解.在维持期间，两名冠状动脉周炎患者经历了临床复发。
结论：由于许多患者无症状，IgG4-RD的心脏受累很少且容易被忽视，诊断依赖于成像。患者对基于糖皮质激素的治疗表现出令人满意的反应。
方法：我们进行了一项回顾性研究，从北京协和医院和北京安贞医院的IgG4-RD队列中纳入了42例IgG4-RD患者，从2010年到2022年。临床,实验室,收集放射学数据，并分析了对糖皮质激素和免疫抑制剂的治疗反应。
结果：与IgG4相关的心脏受累是IgG4-RD谱的罕见部分。冠状动脉周围炎和心包炎的发生率分别为1.2%(13/1075)和3.1%(33/1075),分别在我们的队列中。在两名患者中检测到可能与IgG4-RD相关的瓣膜疾病。没有发现心肌受累的患者。平均年龄58.2±12.8岁,男性占主导地位（76.7%）。冠状动脉CT显示，肿块样和弥漫性壁增厚病变是最常见的冠状动脉周炎类型。心包炎表现为心包积液，局部增厚,钙化和肿块。糖皮质激素和免疫抑制剂治疗后，所有患者的IgG4-RD反应者指数评分均降低,并获得放射学缓解.在维持期间，两名冠状动脉周炎患者经历了临床复发。
结论：由于许多患者无症状，IgG4-RD的心脏受累很少且容易被忽视，诊断依赖于成像。患者对基于糖皮质激素的治疗表现出令人满意的反应。
