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Peer-Reviewed Publication
JACC Case Rep2026;31(14):107264.April 8, 2026Case Reports

IgG4-Related Disease Presenting With Diffuse Coronary Atherosclerosis Characterized by Marked Lipid-Rich Necrotic Core Burden.

Francisco X Elisarraras1, Robert A Pelberg2, Jacob S Roberts3, Jay A Shah4, Jeffrey Xia4, Sammy Sayed4, Norman E Lepor5, Jairo Aldana-Bitar2, Ronald P Karlsberg6, Geoffrey Cho7
1University of California-Los Angeles Health, Los Angeles, California, USA. Electronic address: felisarraras@mednet.ucla.edu.
2Elucid Bioimaging, Boston, Massachusetts, USA.
3Division of Cardiology, Johns Hopkins Hospital, Baltimore, Maryland, USA.
4University of California-Los Angeles Health, Los Angeles, California, USA.
5National Heart Institute, Beverly Hills, California, USA; Cedars-Sinai Heart Institute, Los Angeles, California, USA.
6Cedars-Sinai Heart Institute, Los Angeles, California, USA; Cardiovascular Research Foundation of Southern California, Los Angeles, California, USA; David Geffen School of Medicine at the University of California-Los Angeles, Los Angeles, California, USA.
7University of California-Los Angeles Health, Los Angeles, California, USA; Cardiovascular Research Foundation of Southern California, Los Angeles, California, USA; David Geffen School of Medicine at the University of California-Los Angeles, Los Angeles, California, USA.

Abstract

BACKGROUND: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder with multiorgan involvement, including increasingly recognized cardiovascular manifestations such as aortitis and periarteritis. Coronary involvement remains rare and is often clinically silent and poorly characterized, with most reported cases associated with pericoronary inflammation, ectasia, or aneurysmal dilat…

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