Bussinguer Michelle, Danielian Alfred, Sharma Om P
Division of Pulmonary and Critical medicine, Keck School of Medicine of USC, Los Angeles, CA, USA,
Curr Treat Options Cardiovasc Med. 2012 Dec;14(6):652-64. doi: 10.1007/s11936-012-0208-3.
Cardiac sarcoidosis can be life threatening if not promptly diagnosed and treated appropriately and aggressively. The diagnosis of myocardial disease is often difficult and circumstantial because there are no reliable diagnostic tests. Except for the finding of noncaseating granulomas on endomyocardial biopsy, most tests are limited and nonspecific. Therefore, the decision of initiating treatment is based on the patient's symptoms and the course of the disease, rather than on the presence of histologic confirmation. The goal of therapy is to prevent irreversible cardiomyopathy and to thwart the progression to heart transplantation. The mainstay of treatment is corticosteroids, although there are no large randomized trials analyzing corticosteroid use. The combination with other immunosuppressant agents, such as Methotrexate and Azathioprine, is initiated on the patient's failing or experiencing severe side effects from corticosteroids. While there are small studies proving the efficacy of tumor necrosis factor-inhibitors in cardiac sarcoidosis, more experience with these agents is needed. Catheter ablation or placement of implantable devices is indicated prophylactically in patients with severe ventricular tachyarrhythmias. Heart transplantation should be considered in patients with severe heart failure refractory to medical therapy. This article focuses on the current diagnostic tests and treatment recommendations for cardiac sarcoidosis.
心脏结节病若不及时诊断并给予恰当积极的治疗，可能会危及生命。心肌病的诊断往往困难且需依据具体情况，因为尚无可靠的诊断测试。除了心内膜活检发现非干酪样肉芽肿外，大多数检查都有局限性且缺乏特异性。因此，启动治疗的决策基于患者的症状和疾病进程，而非组织学确诊情况。治疗的目标是预防不可逆的心肌病，并阻止病情发展至心脏移植。治疗的主要手段是使用皮质类固醇，尽管尚无大型随机试验分析皮质类固醇的使用情况。当患者对皮质类固醇治疗无效或出现严重副作用时，可加用其他免疫抑制剂，如甲氨蝶呤和硫唑嘌呤。虽然有小型研究证明肿瘤坏死因子抑制剂对心脏结节病有效，但还需要更多关于这些药物的经验。对于有严重室性快速心律失常的患者，预防性地进行导管消融或植入可植入设备。对于药物治疗难治的严重心力衰竭患者，应考虑心脏移植。本文重点介绍了心脏结节病目前的诊断测试和治疗建议。
