Interstitial Lung Disease Unit.
Cardiology Department, Royal Brompton Hospital, London, UK.
Curr Opin Pulm Med. 2019 Sep;25(5):519-525. doi: 10.1097/MCP.0000000000000611.
In sarcoidosis, the appropriate management strategy remains challenging especially because of the lack of confident diagnosis, considerable variability in initial presentation, disease evolution, and outcome. Although asymptomatic patients with limited cardiac involvement have been described to have a benign outcome, cardiac sarcoidosis is associated with high morbidity and mortality and even sudden cardiac death in a significant proportion of patients. Higher morbidity and mortality can be related with both the disease activity and extent of fibrosis.
Historical series suggested a 5-year mortality rate of 60% in patients with cardiac sarcoidosis. This has definitely improved with the appropriate use of anti-inflammatory medications as well as heart failure treatment, antiarrhythmic medication and device implantation. Timely recognition and vigorous initial approach is essential in avoiding life-threatening arrhythmias and sudden cardiac death. Advanced imaging modalities have proven to be helpful in the diagnostic approach and guiding treatment decisions. However, there is no optimal screening and risk stratification strategy available and further studies are required to determine, which patients would benefit from the available treatments.
This review concentrates on the broad principles of management in cardiac sarcoidosis and the efficacy of sarcoidosis-specific medication and cardiac-specific therapies for cardiac dysfunction and rhythm disturbances.
在结节病中,适当的管理策略仍然具有挑战性,特别是因为缺乏明确的诊断、初始表现、疾病进展和结果的显著变异性。尽管已经描述了无症状且心脏受累有限的患者具有良性结局,但心脏结节病与相当一部分患者的高发病率和死亡率甚至心源性猝死相关。更高的发病率和死亡率可能与疾病活动度和纤维化程度有关。
历史系列表明,心脏结节病患者的 5 年死亡率为 60%。随着抗炎药物以及心力衰竭治疗、抗心律失常药物和器械植入的合理应用,这一数据肯定有所改善。及时识别和积极的初始方法对于避免危及生命的心律失常和心源性猝死至关重要。先进的成像方式已被证明有助于诊断方法,并指导治疗决策。然而,目前尚无最佳的筛查和风险分层策略,需要进一步研究确定哪些患者将从现有治疗中受益。
本综述集中讨论了心脏结节病管理的一般原则,以及结节病特异性药物和心脏特异性治疗在心脏功能障碍和节律紊乱中的疗效。