Performance of diagnostic criteria in patients clinically judged to have cardiac Sarcoidosis: Is it time to regroup?

Abstract Background The diagnosis of cardiac sarcoidosis (CS) is challenging. Due to the current limitations of endomyocardial biopsy as a reference standard, physicians rely on advanced cardiac imaging, multidisciplinary evaluation, and diagnostic criteria to diagnose CS. Aims To compare the three main available diagnostic criteria in patients clinically judged to have CS. Methods We prospectively included patients clinically judged to have cardiac sarcoidosis by a multidisciplinary sarcoidosis team from November 2016 to October 2017. We included only incident cases (diagnosis of CS within one year of inclusion). We applied retrospectively the following diagnostic criteria: the World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG), the Heart Rhythm Society (HRS), and the Japanese Circulation Society (JCS) 2016 criteria. Results We identified 69 patients. Diagnostic criteria classified patients as follows: WASOG as highly probable (1.4%), probable (52.2%), possible (0%), some criteria (40.6%), and no criteria (5.8%); HRS as histological diagnosis (1.4%), probable (52.2%), some criteria (40.6%), and no criteria (5.8%); JCS as histological diagnosis (1.4%), clinical diagnosis (58%), some criteria (39.1%), and no criteria (1.4%). Concordance was high between WASOG and HRS (kappa = 1), but low between JCS and the others (kappa = 0.326). Conclusion A high proportion of patients clinically judged to have CS are unable to be classified according to the three main diagnostic criteria. There is low concordance between JCS criteria and the other two criteria (WASOG and HRS).