Utility of Updated Japanese Circulation Society Guidelines to Diagnose Isolated Cardiac Sarcoidosis

Abstract

Background In the population with cardiac sarcoidosis (CS), approximately one third lacks extracardiac involvement and is considered to have isolated CS. Recently, the Japanese Circulation Society updated the diagnostic criteria for CS, providing a methodology for diagnosing isolated CS. We aimed to assess the characteristics of isolated CS diagnosed using a multimodal imaging approach according to the updated Japanese Circulation Society guidelines. Methods and Results We retrospectively identified 161 consecutive patients who underwent 18F‐fluorodeoxyglucose positron emission tomography for suspected CS between 2012 and 2019. According to the guidelines, patients were classified as having CS with extracardiac involvement, isolated CS, or no CS. We compared the characteristics of multimodality imaging and the prevalence of major adverse cardiovascular events. The Japanese Circulation Society criteria classified 28 patients (17%) as having CS with 4 (2%) with histological confirmation, 21 (13%) as isolated CS, and 112 (70%) as no CS. Compared with CS, isolated CS showed higher left ventricular volume and reduced left ventricular ejection fraction ( P <0.01 for all). During the median follow‐up period of 522 days, 24 patients had major adverse cardiovascular events. Isolated CS (hazard ratio, 3.35; [95% CI, 1.08–10.39], P =0.036) was independently associated with major adverse cardiovascular events after adjusting for reduced left ventricular ejection fraction and steroid. In the subgroup of 41 patients with serial 18F‐fluorodeoxyglucose positron emission tomography evaluation, only updated CS criteria were associated with improvement in myocardial inflammation on 18F‐fluorodeoxyglucose positron emission tomography. Conclusions Isolated CS detected using the updated Japanese Circulation Society guidelines was associated with poor event‐free survival and should be managed with caution.

Unexpected Case of Cardiac Sarcoidosis in a Caucasian Male

“…Patients with diagnosed or suspected CS typically receive a three-day pulse of intravenous methylprednisolone followed by prednisone 40 mg/day for a minimum of four weeks [ 12 ]. Given the high risks of sudden cardiac death, patients with severe arrhythmia or AV block should also be considered for ICD placement in addition to medical therapy; this is classified as a class IIa recommendation by the 2014 HRS guidelines and is supported by additional studies regarding CS patients with advanced conduction system abnormalities [ 6 , 8 , 14 ]. Interestingly, two cases were found in which AV block appeared to be reversed by corticosteroid therapy alone.…”

“…The diagnosis of CS is guided by three major societies: the Heart Rhythm Society 2014 [ 5 ], Japanese Circulation Society 2017 [ 6 ], and World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) 2014 [ 7 ]. While histological confirmation offers the highest level of certainty, the gold standard for diagnosis, endomyocardial biopsy (EMB), still carries limited sensitivity that is estimated to be around 25% [ 8 ].…”

Diagnosis and Treatment of Cardiac Sarcoidosis

“…6 Conduction abnormalities, ventricular arrhythmias (VAs), including sudden cardiac death (SCD), and heart failure (HF), are the presenting features of clinically manifest CS (Table 1). 7,8,10- 13 SCD may be the first manifestation of CS. 10, 14 In a recent investigation of 351 CS patients, 54 of 84 deaths recorded (64%) were unexpected SCD in which CS was diagnosed only postmortem.…”

“…Very importantly and for the first time, the updated JCS guidelines propose diagnostic criteria for isolated CS. 13,26 Recent data suggest that clinically isolated CS may be more aggressive, with nearly double the rate (67% vs. 38%) of adverse outcomes than in CS with symptoms in other organs. 13,27 However, it must be appreciated that these results may reflect significant selection bias, because many patients diagnosed with systemic CS are often asymptomatic and identified by screening.…”

“…13,26 Recent data suggest that clinically isolated CS may be more aggressive, with nearly double the rate (67% vs. 38%) of adverse outcomes than in CS with symptoms in other organs. 13,27 However, it must be appreciated that these results may reflect significant selection bias, because many patients diagnosed with systemic CS are often asymptomatic and identified by screening. Clinically isolated CS, on the other hand, implies typical cardiac presentations/symptoms.…”