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Isolated cardiac sarcoidosis: A focused review of an under-recognized entity #MMPMID27613395
Okada DR; Bravo PE; Vita T; Agarwal V; Osborne MT; Taqueti VR; Skali H; Chareonthaitawee P; Dorbala S; Stewart G; Di Carli M; Blankstein R
J Nucl Cardiol 2018[Aug]; 25 (4): 1136-46 PMID27613395show ga
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.