Division of Rheumatology, Department of Internal Medicine, Toho University Sakura Medical Center, Sakura, Japan.
Division of Respiratory Medicine, Department of Internal Medicine, Toho University Sakura Medical Center, Sakura, Japan.
Medicine (Baltimore). 2023 Aug 4;102(31):e34533. doi: 10.1097/MD.0000000000034533.
Although 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.
A 65-year-old woman was referred to our hospital for chest discomfort and back pain.
In 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.
An 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.
She remains under observation without disease recurrence.
IgG4-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.
尽管 IgG4 相关疾病（IgG4-RD）可影响多种器官，但它与心脏肿块的关联极为罕见。在此，我们报告了 1 例 IgG4-RD 合并心脏肿块的女性病例，并讨论了之前报道的 10 例类似病例。
一名 65 岁女性因胸痛和背痛被转至我院。
根据 2019 年 ACR/EULAR IgG4-RD 诊断标准，根据组织病理学上的密集淋巴细胞浸润、免疫组化染色上 IgG/IgG4 阳性细胞比例<40%、>10/hpf IgG4 阳性细胞、以及胸部椎旁区软组织病变，该患者被诊断为 IgG4-RD。
心电图示完全性房室传导阻滞，予植入体外起搏器。IgG4-RD 诊断明确后，予糖皮质激素和利妥昔单抗治疗。
目前患者仍在观察中，无疾病复发。
IgG4-RD 通常采用糖皮质激素治疗；然而，对于心脏肿块患者，可能发生危及生命的并发症，通常需要手术。糖皮质激素和利妥昔单抗联合治疗对 IgG4-RD 合并心脏肿块患者可能有效，从而避免了侵袭性治疗（如手术）的需要。
