Department of Cardiovascular Medicine, University of Nebraska Medical Center, Omaha, NE.
Department of Cardiology, Cardiovascular Imaging Institute, The Methodist DeBakey Heart and Vascular Center, Houston, TX.
Am J Ther. 2019 Sep/Oct;26(5):600-603. doi: 10.1097/MJT.0000000000000796.
Giant cell myocarditis (GCM) is a rare and a rapidly progressive disorder with fatal outcomes such that patients often require heart transplantation. We present a case of recurrent GCM in a transplanted patient with a history of Crohn disease requiring a novel therapeutic approach.
After the orthotopic heart transplantation, GCM recurred on aggressive immunosuppression over the months, which included corticosteroids, basiliximab, tacrolimus, antithymocyte globulin, and rituximab. Although combination immunosuppressive therapy containing cyclosporine and 2-4 additional drugs including corticosteroids, azathioprine, mycophenolate mofetil, muromonab, gammaglobulin, or methotrexate have shown to prolong the transplant-free survival by keeping the disease under control, its role in preventing and treating recurrence posttransplantation is unclear.
We added sirolimus, a macrolide antibiotic, with properties of T- and B-lymphocyte proliferation inhibition on the above immunosuppressive treatment postrecurrence of GCM. After sirolimus initiation and continuation, the patient has remained disease free.
巨细胞心肌炎（GCM）是一种罕见且快速进展的疾病，其结局往往是致命的，因此患者通常需要进行心脏移植。我们报告了一例患有克罗恩病病史的移植后患者反复发作 GCM 的病例，需要采用新的治疗方法。
在原位心脏移植后，GCM 在数月的强化免疫抑制治疗下复发，包括皮质类固醇、巴利昔单抗、他克莫司、抗胸腺细胞球蛋白和利妥昔单抗。尽管包含环孢素和另外 2-4 种药物的联合免疫抑制治疗（包括皮质类固醇、硫唑嘌呤、霉酚酸酯、莫罗单抗、丙种球蛋白或甲氨蝶呤）已被证明通过控制疾病来延长移植后无病生存时间，但在预防和治疗移植后复发方面的作用尚不清楚。
我们在 GCM 复发后在上述免疫抑制治疗的基础上加用西罗莫司，这是一种大环内酯类抗生素，具有抑制 T 和 B 淋巴细胞增殖的特性。西罗莫司开始和持续使用后，患者疾病未再发作。
