Question:

Which of the following is FALSE regarding immunoglobulin G4-related coronary periarteritis?
1. It may appear as large nodular lesions that cause mass effect on surrounding tissues.
2. It may appear as diffuse thickening of coronary vessel walls.
3. The degree of vessel wall thickening correlates with severity of symptoms of coronary ischemia.
4. Both aneurysmal dilation and stenosis of vessels are sequelae of immunoglobulin G4-related coronary periarteritis.
5. Elevated serum immunoglobulin G4 is neither necessary nor sufficient for diagnosis.





Answer:

The correct answer for the question "Which of the following is FALSE regarding immunoglobulin G4-related coronary periarteritis?" is:

3. The degree of vessel wall thickening correlates with severity of symptoms of coronary ischemia.



Explanation
A. True. [IgG4-related coronary periarteritis may manifest as: 1) one or more homogeneous nodular lesions, or pseudotumors, along the coronary artery walls or 2) rinds of soft-tissue attenuation surrounding the coronary arteries.] [Echocardiography was not obtained but may detect large coronary periarterial lesions, evidence of mass effect on nearby structures, and impairment of left ventricular motion and function in the setting of ischemia.]
B. True. [IgG4-related coronary periarteritis may manifest as: 1) one or more homogeneous nodular lesions, or pseudotumors, along the coronary artery walls or 2) rinds of soft-tissue attenuation surrounding the coronary arteries.]
C. False. [The presence of ischemia does not seem to correlate with degree of vessel wall thickening.]
D. True. [Coronary artery stenosis, ectasia, and aneurysm/pseudoaneurysm formation have been reported in association with IgG4-related coronary periarteritis.]
E. True. [Elevated serum IgG4 levels are neither necessary nor sufficient for diagnosis, as in our case.]



From the manuscript:
Coronary periarteritis in a patient with multi-organ IgG4-related disease
Radiology Case. 2015 Jan; 9(1):1-17


This article belongs to the Cardiac section.




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From the manuscript

Coronary periarteritis in a patient with multi-organ IgG4-related disease

Free full text article: Coronary periarteritis in a patient  with multi-organ IgG4-related disease

Abstract
Immunoglobulin G4-related disease is a recently described systemic clinicopathological entity characterized by immunoglobulin G4-producing plasmacytic infiltration of tissue and frequently by elevated serum immunoglobulin G4 concentration. Manifestations of this disease have been documented in nearly all organs and locations, but coronary artery involvement is not widely recognized. We report the coronary findings of a patient with multi-organ immunoglobulin G4-related disease. Non-electrocardiogram-gated computed tomography of the chest demonstrated nodular and rind-like periarterial soft tissue thickening along the proximal coronary artery segments with improvement following steroid therapy.






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