Table 2.
WASOG organ-assessment tool126 | Expert-consensus recommendations on criteria for the diagnosis of cardiac sarcoidosis90 |
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Highly probable N/A At least probable – Treatment-responsive cardiomyopathy or atrioventricular nodal block – Reduced left ventricular ejection fraction in the absence of other clinical risk factors – Spontaneous or inducible sustained ventricular tachycardia with no other risk factor – Mobitz type II or third-degree heart block – Patchy uptake on dedicated cardiac positron-emission tomography – Delayed enhancement on cardiac magnetic resonance (CMR) imaging – Positive gallium uptake – Defect on perfusion scintigraphy or SPECT scan – T2 prolongation on CMR Possible – Reduced left ventricular ejection fraction in the presence of other risk factors (eg, hypertension, diabetes mellitus) – Atrial dysrhythmias No consensus – Frequent ectopia (>5% QRS complexes) – Bundle-branch block – Impaired right ventricular function with normal pulmonary vascular resistance – Fragmented QRS or pathologic Q waves in two or more anatomically contiguous leads – At least one abnormal signal-averaged electrocardiogram – Interstitial fibrosis or monocyte infiltration |
1. Histological diagnosis from myocardial tissue cardiac sarcoidosis is performed in the presence of noncaseating granuloma on histological examination of myocardial tissue, with no alternative cause identified (including negative organismic stains if applicable) 2. Clinical diagnosis from invasive and noninvasive studies. It is probable that cardiac sarcoidosis is present if: a) there is histological diagnosis of extracardiac sarcoidosis, and b) one or more of the following is present: ▪ corticosteroid-responsive and/or immunosuppressant-responsive cardiomyopathy or heart block ▪ unexplained reduced left ventricular ejection fraction (<40%) ▪ unexplained sustained (spontaneous or induced) ventricular tachycardia ▪ Mobitz II second-degree heart block or third-degree heart block ▪ patchy uptake on dedicated cardiac positron-emission tomography (in a pattern consistent with cardiac sarcoidosis) ▪ late gadolinium enhancement on cardiovascular magnetic resonance (in a pattern consistent with cardiac sarcoidosis) ▪ positive gallium uptake (in a pattern consistent with cardiac sarcoidosis), and c) other causes for cardiac manifestation(s) have been reasonably excluded |
Notes: Adapted from Consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Birnie DH, Sauer WH, Judson MA. 102(6):411–414. Heart. Copyright © 2016 with permission from BMJ Publishing Group Ltd.90 Adapted from Judson MA, Costabel U, Drent M, et al. The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(1):19–27.126
Abbreviations: SPECT, single-photon emission computed tomography; WASOG, World Association of Sarcoidosis and Other Granulomatous Diseases.