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Clinical Need: Current Assessment and Supportive Data

Sarcoidosis is a multisystem inflammatory disease of unknown etiology that affects young men and women, typically before the age of 50 years.1 The incidence of sarcoidosis varies based on a variety of factors including race, gender, and geography with incidence ranging from 0.73 per 100,000 in Japanese males,2 10 per 100,000 age- and gender-adjusted in a predominantly white cohort in the United States1 and 71 per 100,000 in African American females.3

Cardiac sarcoidosis (CS) has been reported to involve approximately 2%-5% of patients with systemic sarcoidosis.1 It can also be the only manifestation of sarcoidosis. The clinical presentation is nonspecific and includes sudden cardiac death, ventricular arrhythmias, progressive heart failure, and conduction disturbances. Despite its low reported prevalence, CS accounts for 13%-25% of sarcoidosis-related deaths.4

Current diagnostic criteria for CS include the Japanese Ministry of Health and Welfare (JMWH) criteria5 and the Heart Rhythm Society Consensus (HRS).6 The gold standard for CS is endomyocardial biopsy, which has a low diagnostic yield owing to heterogeneous and patchy involvement of the myocardium. 2-Deoxy-2-[F-18]Fluoro-D-glucose (18F-FDG) PET-CT is the best advanced imaging tool to evaluate myocardial inflammation in patients with suspected CS. It has a reported sensitivity of up to 85%-100% with more variable specificity of 39%-100%.7–10 The low specificity is likely due in part to using the JMHW criteria as a gold standard and the difficulty of a definitive diagnosis.

In the HRS guidelines,6 clinical diagnosis of CS is probable if there is histological proof of extra CS and one or more advanced imaging findings of CS or unexplained decreased systolic function or arrhythmias when other causes have been reasonably excluded (Table 12-1).

Table 12-1Diagnostic Criteria for Cardiac Sarcoidosis

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