Dominati Arnaud, Urbanski Geoffrey, Meyer Philippe, Seebach Jörg D
Division of Clinical Immunology and Allergology, Department of Medicine, Geneva University Hospital, 1205 Geneva, Switzerland.
Division of Rheumatology, Department of Medicine, University of Illinois College of Medicine in Chicago, Chicago, IL 60612, USA.
J Clin Med. 2025 Sep 3;14(17):6234. doi: 10.3390/jcm14176234.
Cardiac sarcoidosis (CS) is a granulomatous inflammatory cardiomyopathy with heterogeneous presentations, from palpitations to heart failure and sudden cardiac arrest. Despite advances in imaging and immunosuppressive (IS) therapy, relapse patterns and long-term outcomes remain poorly defined. This study aimed to characterize relapse and identify predictors of relapse and major adverse cardiac events (MACE) in a real-world CS cohort. This retrospective single-center study included 25 adults diagnosed with CS at Geneva University Hospitals between 2016 and 2024, classified per the 2024 American Heart Association diagnostic criteria. Relapse was defined as clinical, arrhythmic, or imaging deterioration requiring treatment escalation. MACE included cardiovascular hospitalization, device therapy, left ventricular assist device, heart transplant, or death. Statistical methods included Kaplan-Meier analysis with log-rank tests and multivariable Cox regression adjusted for age and sex. Relapse occurred in 13 patients (56%), frequently subclinical (61.5%) and detected incidentally on routine PET-CT during IS tapering. In the multivariate model, predictors of relapse included right ventricular FDG uptake (aHR 13.1; 95% CI 1.3-133.7; = 0.03) and second-line immunosuppression duration ≤24 months (aHR 20.1; 95% CI 1.1-363.8; = 0.04). Relapse-free patients were more often maintained on dual or triple IS therapy (71.4% vs. 15.4%; = 0.02) and low-dose prednisone (<10 mg/day) (57.1% vs. 7.7%; = 0.03). Relapse is common in CS, often subclinical, and associated with PET-CT findings and premature IS tapering. Maintenance therapy may reduce risk. Multimodal imaging remains critical for disease monitoring, though tracers with higher specificity are needed. Further research should refine relapse definitions and support personalized treatment strategies.
心脏结节病(CS)是一种肉芽肿性炎症性心肌病,表现多样,从心悸到心力衰竭和心搏骤停。尽管在影像学和免疫抑制(IS)治疗方面取得了进展,但复发模式和长期预后仍不明确。本研究旨在描述真实世界中CS队列的复发情况,并确定复发和主要不良心脏事件(MACE)的预测因素。这项回顾性单中心研究纳入了2016年至2024年期间在日内瓦大学医院被诊断为CS的25名成年人,根据2024年美国心脏协会诊断标准进行分类。复发定义为需要升级治疗的临床、心律失常或影像学恶化。MACE包括心血管住院、器械治疗、左心室辅助装置、心脏移植或死亡。统计方法包括采用对数秩检验的Kaplan-Meier分析和根据年龄和性别进行调整的多变量Cox回归。13名患者(56%)出现复发,复发常为亚临床型(61.5%),在IS减量期间的常规PET-CT检查中偶然发现。在多变量模型中,复发的预测因素包括右心室FDG摄取(调整后风险比[aHR] 13.1;95%置信区间[CI] 1.3 - 133.7;P = 0.03)和二线免疫抑制持续时间≤24个月(aHR 20.1;95% CI 1.1 - 363.8;P = 0.04)。无复发患者更常接受双联或三联IS治疗(71.4%对15.4%;P = 0.02)和低剂量泼尼松(<10 mg/天)(57.1%对7.7%;P = 0.03)。复发在CS中很常见,常为亚临床型,与PET-CT结果和过早的IS减量有关。维持治疗可能降低风险。多模态成像对于疾病监测仍然至关重要,尽管需要特异性更高的示踪剂。进一步的研究应完善复发定义并支持个性化治疗策略。