Abstract
Sarcoidosis is a chronic systemic disease of unknown etiology that is characterized by the presence of noncaseating epithelioid granulomas, usually in multiple organs. Several studies have shown that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals. Cardiac involvement may occur and lead to an adverse outcome: the heart mechanics will be affected and that causes ventricular failure, and the cardiac electrical system will be disrupted and lead to third degree atrioventricular block, malignant ventricular tachycardia, and sudden cardiac death. Thus, early diagnosis and treatment of this potentially devastating disease is critically important. However, sensitive and accurate imaging modalities have not been established. Recent studies have demonstrated the promising potential of cardiac magnetic resonance imaging (MRI) and 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET) in the diagnosis and assessment of cardiac sarcoidosis (CS). In this review, we discuss the epidemiology, etiology, histological findings, and clinical features of sarcoidosis. We also introduce advanced imaging including 18F-FDG PET and cardiac MRI as more reliable diagnostic modalities for CS.
Highlights
Sarcoidosis was first reported more than 120 years ago
The etiology of sarcoidosis has not been elucidated, most evidence points to an aberrant immune response as the pathogenetic mechanism, which is driven by an unidentified antigen in genetically susceptible individuals
Granulomas typically consist of a compact central area of macrophages that differentiate into epithelioid cells and fuse to form multinucleated giant cells surrounded by lymphocytes (Figure 1) [3]
Summary
Sarcoidosis was first reported more than 120 years ago It is a granulomatous inflammatory disease with an unclear etiology that affects multiple organs including the lungs, heart, skin, central nervous system, and eyes [1]. It is not commonly fatal, cardiac involvement may be responsible for more than two-thirds of deaths [2]. Once the diagnosis of sarcoidosis is made, management may range from observation to long-term administration of steroids (often at high doses) or other immunosuppressive therapies, depending on disease severity and organ involvement [3]. The diagnosis of CS is further hindered by the lack of any reliable and specific diagnostic test, as there are no imaging findings that allow for a definitive diagnosis of CS
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