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Isolated cardiac sarcoidosis: A focused review of an under-recognized entity.

ABSTRACT: There is accumulating evidence for the existence of a phenotype of isolated cardiac sarcoidosis (ICS), or sarcoidosis that only involves the heart. In the absence of biopsy-confirmed cardiac sarcoidosis (CS), existing diagnostic criteria require the presence of extra-cardiac sarcoidosis as an inclusion criterion for the diagnosis of CS. Consequently, in the absence of a positive endomyocardial biopsy, ICS is not diagnosable by current guidelines. Therefore, there is uncertainty regarding the epidemiology, pathobiology, clinical characteristics, prognosis, and optimal treatment of ICS. This review will summarize the available data related to the prevalence and prognosis of ICS and will discuss challenges surrounding the diagnosis and management of this under-recognized entity.

SUBMITTER: Okada DR 

PROVIDER: S-EPMC5540795 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature

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Isolated cardiac sarcoidosis: A focused review of an under-recognized entity.

Okada David R DR   Bravo Paco E PE   Vita Tomas T   Agarwal Vikram V   Osborne Michael T MT   Taqueti Viviany R VR   Skali Hicham H   Chareonthaitawee Panithaya P   Dorbala Sharmila S   Stewart Garrick G   Di Carli Marcelo M   Blankstein Ron R  

Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology 20160909 4

There is accumulating evidence for the existence of a phenotype of isolated cardiac sarcoidosis (ICS), or sarcoidosis that only involves the heart. In the absence of biopsy-confirmed cardiac sarcoidosis (CS), existing diagnostic criteria require the presence of extra-cardiac sarcoidosis as an inclusion criterion for the diagnosis of CS. Consequently, in the absence of a positive endomyocardial biopsy, ICS is not diagnosable by current guidelines. Therefore, there is uncertainty regarding the epi  ...[more]

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