Naseeb Muhammad Wahdan, Adedara Victor O, Haseeb Muhammad Talha, Fatima Hareem, Gangasani Swapna, Kailey Kamaljit R, Ahmed Moiz, Abbas Kiran, Razzaq Waleed, Qayyom Muhammad M, Abdin Zain U
Internal Medicine, Dow University of Health Sciences, Karachi, PAK.
Medicine, St. George's University School of Medicine, St. George's, GRD.
Cureus. 2023 Jun 14;15(6):e40439. doi: 10.7759/cureus.40439. eCollection 2023 Jun.
Giant cell myocarditis (GCM) is a rare, often rapidly progressive, and potentially fatal disease because of myocardium inflammation due to the infiltration of giant cells triggered by infectious as well as non-infectious etiologies. Several studies have reported that GCM can occur in patients of all ages but is more commonly found in adults. It is relatively more common among African American and Hispanic patients than in the White population. Early diagnosis and treatment are critical. Electrocardiogram (EKG), complete blood count, erythrocyte sedimentation rate, C-reactive protein, and cardiac biomarkers such as troponin and brain natriuretic peptide (BNP), echocardiogram, cardiac magnetic resonance imaging (MRI), myocardial biopsy, and myocardial gene profiling are useful diagnostic tools. Current research has identified several potential biomarkers for GCM, including myocarditis-associated immune cells, cytokines, and other chemicals. The standard of care for GCM includes aggressive immunosuppressive therapy with corticosteroids and immunomodulatory agents like rituximab, cyclosporine, and infliximab, which have shown promising results in GCM by balancing the immune system and preventing the attack on healthy tissues, resulting in the reduction of inflammation, promotion of healing, and decreasing the necessity for cardiac transplantation. Without immunosuppression, the chance of mortality or cardiac surgery was 100%. Multiple studies have revealed that a treatment combination of corticosteroids and immunomodulatory agents is superior to corticosteroids alone. Combination therapy significantly increased transplant-free survival (TFS) and decreased the likelihood of heart transplantation, hence improving overall survival. It is important to balance the benefits of immunosuppression with its potentially adverse effects. In conclusion, immunomodulatory therapy adds significant long-term survival benefits to GCM.
巨细胞性心肌炎(GCM)是一种罕见的、通常进展迅速且可能致命的疾病,其病因是感染性和非感染性因素引发巨细胞浸润,进而导致心肌炎症。多项研究报告称,GCM可发生于各年龄段患者,但在成年人中更为常见。在非裔美国人和西班牙裔患者中相对比白人更为常见。早期诊断和治疗至关重要。心电图(EKG)、全血细胞计数、红细胞沉降率、C反应蛋白以及心肌生物标志物如肌钙蛋白和脑钠肽(BNP)、超声心动图、心脏磁共振成像(MRI)、心肌活检和心肌基因谱分析都是有用的诊断工具。目前的研究已确定了几种GCM的潜在生物标志物,包括与心肌炎相关的免疫细胞、细胞因子和其他化学物质。GCM的标准治疗包括使用皮质类固醇和免疫调节药物如利妥昔单抗、环孢素和英夫利昔单抗进行积极的免疫抑制治疗,这些药物通过平衡免疫系统和防止对健康组织的攻击,在GCM中显示出了有前景的结果,从而减少炎症、促进愈合并降低心脏移植的必要性。若无免疫抑制,死亡率或心脏手术的几率为100%。多项研究表明,皮质类固醇和免疫调节药物联合治疗优于单独使用皮质类固醇。联合治疗显著提高了无移植生存率(TFS)并降低了心脏移植的可能性,从而改善了总体生存率。平衡免疫抑制的益处与其潜在的不良反应很重要。总之,免疫调节治疗为GCM带来了显著的长期生存益处。