Elsevier

American Heart Journal

Volume 223, May 2020, Pages 106-109
American Heart Journal

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

https://doi.org/10.1016/j.ahj.2020.02.008Get rights and content

Background

The diagnosis of cardiac sarcoidosis (CS) is challenging. Because of 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 3 main available diagnostic criteria in patients clinically judged to have CS.

Methods

We prospectively included patients clinically judged to have CS by a multidisciplinary sarcoidosis team from November 2016 to October 2017. We included only incident cases (diagnosis of CS within 1 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 (κ = 1) but low between JCS and the others (κ = 0.326).

Conclusions

A high proportion of patients clinically judged to have CS are unable to be classified according to the 3 main diagnostic criteria. There is low concordance between JCS criteria and the other 2 criteria (WASOG and HRS).

Section snippets

Background

The diagnosis of cardiac sarcoidosis (CS) is a very challenging process. Because of the current limitations of endomyocardial biopsy as a reference standard, physicians frequently rely on advanced cardiac imaging, multidisciplinary evaluation, and the available diagnostic criteria to diagnose or rule out CS.1 In the present study, we aimed to compare the performance of the 3 main available diagnostic criteria in patients clinically judged to have CS.

Methods

We prospectively included patients clinically judged to have cardiac sarcoidosis after evaluation in a multidisciplinary sarcoidosis clinic from November 2016 to October 2017. The multidisciplinary evaluation consisted of 2 key components: first, patients were seen in clinic by a pulmonologist and a heart failure specialist cardiologist, both with expertise in sarcoidosis; second, a multidisciplinary discussion was performed including other relevant subspecialties to the case (eg, advance

Results

We identified 69 patients, the majority of whom were male (60.9%) and white (81.2%) (Table I). Most patients (58%) had biopsy proven extracardiac sarcoidosis, with common extracardiac manifestations being pulmonary (69.6%), nonthoracic lymph node (31.9%), splenic (21.7%), and hepatic (14.5%) involvement. Mean (SD) age at CS diagnosis was 53 ± 9.8 years, with median time from symptoms to diagnosis of 7 months (interquartile range 22). CS was suspected based on symptoms (84.1%) or routine

Conclusions

The present study demonstrates that a high proportion of patients clinically judged to have CS by a multidisciplinary evaluation are unable to be classified according to the 3 main diagnostic criteria pathways. Furthermore, it shows a low concordance between the 2016 JCS criteria and the other 2 criteria (WASOG and HRS), with the JCS criteria more frequently classifying patients as having clinical CS compared to WASOG and HRS criteria. These findings are mainly driven by a high proportion of

Acknowledgements

None.

Disclosures

None.

References (14)

There are more references available in the full text version of this article.

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