Vasić Nada, Stević Ruza, Zugić Vladimir, Jovanović Dragana
Klinicki Centar Srbije, Beograd, Institut za plućne bolesti i tuberkulozu.
Med Pregl. 2005;58 Suppl 1:31-4.
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. Clinical manifestations depend on the disease activity and organ involvement. The actual incidence of myocardial sarcoidosis has never been established. Manifestations range from accidental findings and benign conditions to fatal cardiomyopathies and arrhythmias.
Bernstein et al. first described myocardial sarcoidosis in 1929, and Getzen first described a lethal case caused by myocardial sarcoidosis in 1937. A definite histological diagnosis of sarcoidosis is dificult to make, and in some cases treatment should be initiated without histological confirmation. An optimal strategy for establishing diagnosis of myocardial sarcoidosis is not clear. Thallium scintigraphy (with additional use of gallium and technetium) is most often used and investigated 2D echocardiography is less sensitive, but could be an additional tool in diagnostics. New non-invasive techniques, such as magnetic resonance imaging (MRI) and positron emission tomography (PET) can also be applied in diagnostics of this disease. Considering life-threatening potential of myocardial sarcoidosis, prompt and aggressive treatment with corticosteroids and/or inmmunosuppressive drugs is necessary.
Recognition of myocardial sarcoidosis and choice of adequate treatment regimen requires maximal involvement and team work of cardiologists and respirator), specialists. This paper also deals with new diagnostic and therapeutic procedures, because early diagnosis and initiation of adequate treatment are the main prognostic factors of myocardial sarcoidosis.
结节病是一种病因不明的多系统肉芽肿性疾病。临床表现取决于疾病活动度和器官受累情况。心肌结节病的实际发病率尚未确定。其表现范围从偶然发现和良性情况到致命的心肌病和心律失常。
伯恩斯坦等人于1929年首次描述了心肌结节病,格岑于1937年首次描述了一例由心肌结节病导致的致命病例。结节病的确切组织学诊断很难做出,在某些情况下,治疗应在无组织学确诊的情况下开始。建立心肌结节病诊断的最佳策略尚不清楚。铊闪烁扫描(额外使用镓和锝)是最常使用的方法,而经研究二维超声心动图敏感性较低,但可能是诊断中的一种辅助工具。新的非侵入性技术,如磁共振成像(MRI)和正电子发射断层扫描(PET)也可应用于该疾病的诊断。鉴于心肌结节病有危及生命的可能性,使用皮质类固醇和/或免疫抑制药物进行迅速且积极的治疗是必要的。
认识心肌结节病并选择适当的治疗方案需要心脏病专家和呼吸科专家的最大程度参与和团队合作。本文还讨论了新的诊断和治疗方法,因为早期诊断和开始适当治疗是心肌结节病的主要预后因素。