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IgG4-related disease: Coronary arteritis masquerading as coronary “masses”

Published:January 07, 2022DOI:https://doi.org/10.1016/j.jcct.2021.12.009
      A 64-year-old man with a history of psoriasis and latent tuberculosis presented to the emergency department with cough and dyspnea. Transthoracic echocardiogram (TTE) showed a large pericardial effusion with early echocardiographic signs of tamponade and a well circumscribed right atrioventricular groove mass extending into the pericardial space, moving with the cardiac cycle without independent mobility (Fig. 1). Pericardiocentesis drained 1 ​L of sanguinous fluid with a lymphocytic predominance and negative infectious and cytologic work-up. Gated cardiac Computed Tomography Angiography (CTA) demonstrated coronary artery calcifications without obstructive coronary artery disease and two partially vascular hypodense masses encasing the mid right coronary artery (RCA) and the proximal left coronary circulation, suggestive of perivascular arteritis (Fig. 2). Neither mass invaded the cardiac cavities. Initial white blood cell was normal at 6.9 K/μL, with a normal differential (71% neutrophils, 11% lymphocytes, 13% monocytes and 5% eosinophils). C-reactive protein was not evaluated at initial presentation, but was normal at <0.1 mg/dl at 4 months follow up. Additional rheumatologic workup was not performed.
      Fig. 1
      Fig. 1Transthoracic echocardiogram subcostal imaging shows a an echo bright mass at the right atrioventricular groove and a circumferential pericardial effusion.
      Fig. 2
      Fig. 2Cardiac Computer tomography Angiography axial and multiplanar reformatted images demonstrate two partially vascular hypodense areas surrounding the proximal left coronary artery circulation and the mid right coronary artery, consistent with arteritis.

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