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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. 1Transthoracic echocardiogram subcostal imaging shows a an echo bright mass at the
right atrioventricular groove and a circumferential pericardial effusion.
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.
Members of the ACR/EULAR IgG4-RD classification criteria working group. The 2019 American College of Rheumatology/European League against rheumatism classification criteria for IgG4-related disease.
