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Updated: Nov 12, 2025

Identifying Coronary Artery Calcification on Non-gated Computed Tomography Scans
Published on: August 28, 2018
Hidden IgG4-Related Coronary Disease
Namita Ramdin1, Matthew Orde2, Siobhán B O'Neill3
1Arthritis Research Canada, Richmond, Canada.
Insights
This autopsy confirmed IgG4-related coronary arteritis, a rare condition involving inflammation of the coronary arteries. Findings aid in diagnosing and preventing misdiagnosis of coronary artery vasculitis.
Area of Science:
- Cardiovascular Pathology
- Immunopathology
- Radiology
Background:
- Coronary artery vasculitis can be challenging to diagnose.
- Immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized as a systemic fibroinflammatory condition.
Purpose of the Study:
- To present a comprehensive autopsy case with detailed radiologic and pathologic review of coronary arteries.
- To improve diagnostic accuracy and prevent misdiagnosis of coronary artery vasculitis.
Main Methods:
- Full autopsy performed with family consent.
- Histologic processing of cardiac, brain, lung, and spinal cord tissues.
- Cardiac-gated computed tomography coronary angiography.
Main Results:
- Radiography suggested periarteritis with coronary artery ectasia and calcified atherosclerosis.
- Histology revealed dense adventitial fibrosis, lymphoplasmacytic infiltrate, and plasma cells consistent with IgG4-related disease.
- Marked attenuation or absence of the coronary artery media contributed to ectasia.
Conclusions:
- Autopsy confirmed IgG4-related coronary arteritis.
- Coronary periarteritis is an uncommon but established manifestation of IgG4-related disease.
Objectives:
We present a full autopsy with a focused radiology and pathologic review of the coronary arteries. We hope that the results described in this article will help create better diagnostic measures and prevent future coronary artery vasculitis misdiagnosis.
Methods:
A full autopsy was performed on the body of Dr Myung Choong Yoon, with full consent from the family, within the department of pathology and laboratory medicine at Vancouver General Hospital. Tissue samples from the heart, brain, lungs, and spinal cord were submitted to specialist pathologists for histologic processing.
Results:
Cardiac gated computed tomography coronary angiography suggested periarteritis. Coexistent calcified coronary atherosclerosis with linear calcifications was present along the luminal wall, along with coronary artery ectasia. Histologic assessment confirmed features of dense adventitial fibrosis around the coronary arteries, with an exuberant lymphoplasmacytic infiltrate and numerous plasma cells consistent with IgG4-related disease. The media of the coronary arteries was markedly attenuated or completely absent, which likely contributed to the coronary arterial ectasia noted microscopically. These findings confirmed IgG4-related coronary arteritis.
Conclusions:
Coronary periarteritis is an uncommon manifestation of IgG4-related disease established radiographically and later by autopsy.
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