How common is isolated cardiac sarcoidosis? Extra-cardiac and cardiac findings on clinical examination and whole-body 18F–fluorodeoxyglucose positron emission tomography

The Utility of Whole Body 18F-FDG PET-CT in Diagnosing Isolated Cardiac Sarcoidosis: The Western Australian Cardiac Sarcoid Study

“…did not specify the timing of the scan in relation to the diagnosis of sarcoidosis [11]. The authors found that 40% of patients with suspected ICS had clinically silent but radiographically evident ECS after undergoing 18 F-FDG PET-CT. Juneau et al, in 2018, reported an even lower prevalence of 3.2% (1/31) of CS patients with ICS using 18 F-FDG PET-CT imaging combined with clinical assessment to identify extracardiac organ involvement, including dedicated assessment of the eyes and skin at presentation [9]. With the use of highly sensitive whole-body imaging, previously reported rates of ICS may have been overestimated.…”

“…The disparity in reported prevalence is likely related to the variable diagnostic methods for assessing extracardiac involvement. Recent studies indicate a much lower prevalence of ICS with the use of highly sensitive imaging modalities including 18 F-FDG PET-CT, however, no similar study has been previously carried out in an Australian centre [9,10].…”

“…The reported prevalence of CS confined to the heart, known as isolated cardiac sarcoidosis (ICS), varies widely, from 29-52% [3,9]. The disparity in reported prevalence is likely related to the variable diagnostic methods for assessing extracardiac involvement.…”

Cardiac Sarcoidosis

“…Certainly, this seems consistent in the phenotype of primarily Caucasian patients of Northern European descent we and others have described. 1,5,50 Clinical features of CS are dependent on the location, extent, and activity of the disease. The principal manifestations are conduction abnormalities and ventricular arrhythmias including sudden death and heart failure.…”

“…The reported prevalence of isolated CS varies widely with rates from 32 to 54%. 1,5,50,55,56,58 There are three main reasons for this variability. First, the heterogeneous nature of many of the cohorts.…”