Research LetterPerformance of diagnostic criteria in patients clinically judged to have cardiac sarcoidosis: Is it time to regroup?
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.
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Diagnostics, treatment and outcomes of cardiac sarcoidosis in a Norwegian cohort
2024, International Journal of CardiologyCardiac Sarcoidosis
2024, Clinics in Chest MedicineUpdate on cardiac sarcoidosis
2023, Trends in Cardiovascular MedicineCitation Excerpt :Long-term or high dose corticosteroid therapy is associated with numerous adverse side effects including diabetes, cataracts, hypertension, metabolic syndrome, osteoporosis, opportunistic infection, and increased risk of cardiovascular events. Corticosteroid-sparing agents such as methotrexate, mycophenolate mofetil, azathioprine, and leflunomide are often used as second-line or adjuvant agents in the treatment of active cardiac sarcoidosis mostly based on small observational studies [3,6,81]. In a small retrospective study by Rosenthal et al. that studied the efficacy of methotrexate with or without adalimumab in 28 cardiac sarcoidosis patients, steroid-sparing therapies maintained radiographic quiescence after the taper of prednisone in at least 60% patients [85].
Sarcoidosis-Related Cardiomyopathy: Current Knowledge, Challenges, and Future Perspectives State-of-the-Art Review
2022, Journal of Cardiac FailureCitation Excerpt :The diagnosis of CS often requires recognition, and further evaluation, of extracardiac manifestations (Table 3). It is notable that none of the aforementioned diagnostic tools have been prospectively validated in large CS cohorts, resulting in low concordance between diagnostic criteria when applied to patients clinically judged to have CS.38,39 Laboratory tests evaluated as serum biomarkers of CS include angiotensin-converting enzyme, lysozyme, serum soluble IL-2 receptor, and serum/urine calcium.
Cardiac Sarcoidosis: A Clinical Overview
2021, Current Problems in CardiologyCitation Excerpt :There is still a group of patients with clinical suspicion of CS but who do not fall under any of the current guidelines criteria, mainly due to the absence of detectable myocardial inflammation or scarring. This group represents <5% of the patients.49 More research is needed to better define the implications of the changes in diagnostic criteria and potential response to therapy.
Challenges in Cardiac and Pulmonary Sarcoidosis: JACC State-of-the-Art Review
2020, Journal of the American College of CardiologyCitation Excerpt :Lastly, the World Association of Sarcoidosis and Other Granulomatous Diseases developed a sarcoidosis organ assessment instrument that requires histological evidence of granulomatous inflammation (90). A recent study comparing the accuracy of those 3 main diagnostic tools revealed high concordance between World Association of Sarcoidosis and Other Granulomatous Diseases and HRS but low between JCS and the others, raising concerns about the specificity of the diagnosis of sarcoidosis in the absence of confirmatory biopsy (91). The prevalence of cardiac involvement among patients with systemic sarcoidosis screened by advanced imaging has varied widely (3.7% to 54.9%) depending upon the techniques used and the population studied (89).