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Abstract
Sarcoidosis is a chronic inflammatory disease of unknown etiology characterized by multi-organ involvement. End-organ disease consists of granulomatous inflammation, which if left untreated or not resolved spontaneously, leads to permanent fibrosis and end-organ dysfunction. Cardiac involvement and fibrosis in sarcoidosis occur in 5-10% of cases and is becoming increasingly diagnosed. This is due to increased clinical awareness among clinicians and new diagnostic modalities, since magnetic resonance imaging and positron-emission tomography are emerging as "gold standard" tools replacing endomyocardial biopsy. Despite this progress, isolated cardiac sarcoidosis is difficult to differentiate from other causes of arrhythmogenic cardiomyopathy. Cardiac fibrosis leads to congestive heart failure, arrhythmias and sudden cardiac death. Immunosuppressives (mostly corticosteroids) are used for the treatment of cardiac sarcoidosis. Implantable devices like a cardioverter-defibrillator may be warranted in order to prevent sudden cardiac death. In this article current trends in the pathophysiology, diagnosis and management of cardiac sarcoidosis will be reviewed focusing on published research and latest guidelines. Lastly, a management algorithm is proposed.
Highlights
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology manifesting frequently as a mild or even asymptomatic pulmonary disease (Costabel, 2001; Yamamoto et al, 1992)
The diagnosis of Cardiac sarcoidosis (CS) remains a challenge, the evolution of modern imaging modalities such as cardiac magnetic resonance imaging (CMR) and 18-fluorodeoxyglucose positron emission tomography (18F-FDG PET), as well as the application of clinical guidelines have led to increased diagnosis rates (Birnie et al, 2014; Mc Ardle et al, 2013)
implantable cardioverter defibrillators (ICDs) is recommended in case of 1) spontaneous sustained ventricular arrhythmias and 2) in cases of left ventricular ejection fraction (LVEF) < 35% despite optimal immunosuppression treatment
Summary
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology manifesting frequently as a mild or even asymptomatic pulmonary disease (Costabel, 2001; Yamamoto et al, 1992). Cardiac sarcoidosis (CS) is considered to be the second most common cause of death in sarcoidosis patients globally and the first among Japanese sarcoidosis patients (Iwai et al, 1994). Manifest CS occurs in 3-10% of patients with sarcoidosis in America and Europe, though its prevalence from certain autopsy studies is estimated to be up to 25% (Iwai et al, 1993; Perry and Vuitch, 1995; Silverman et al, 1978). CS manifests with ventricular arrhythmia, high grade block, sudden death or symptoms of heart failure most commonly presented with a rather acute onset (Birnie et al, 2016). Almost one third of patients with CS did not have a diagnosis of systemic sarcoidosis (Nery et al, 2014a; Tung et al, 2015). Corticosteroids are considered the standard of care for CS treatment, though there is no consensus regarding the dosage or the duration of treatment and the role of second line steroid-sparing agents (Nagai et al, 2015; Yazaki et al, 2001)
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