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IgG4-related aortitis mimicking acute aortic and coronary syndromes; multimodality imaging–pathology correlation: a case report
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DOI：10.3389/fcvm.2026.1819638.png)
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Abstract
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BackgroundIgG4-related aortitis is an uncommon inflammatory condition that can closely mimic both acute aortic and coronary syndromes; posing a major diagnostic challenge in patients presenting with chest pain.Case summaryA 64-year-old man with prior non–ST-elevation myocardial infarction (NSTEMI) and persistent angina presented with abrupt-onset; severe retrosternal chest pain and dynamic lateral ST-segment depression. Rising high-sensitivity troponin supported a working diagnosis of high-risk NSTEMI. During observation; he developed acute respiratory failure and hemodynamic collapse. Echocardiography revealed a large circumferential pericardial effusion with reduced left ventricular ejection fraction; without classic tamponade physiology. Contrast-enhanced computed tomography demonstrated a crescentic ascending aortic wall thickening consistent with Stanford type A intramural hematoma extending into the arch vessels. A pericardial window drained 550 mL of serohemorrhagic fluid; with transient improvement; but recurrent instability prompted emergent ascending aortic replacement and coronary bypass grafting. Despite maximal support; the patient died intraoperatively. Histopathological analysis revealed dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and storiform fibrosis; confirming IgG4-related aortitis.DiscussionThis case highlights the ability of IgG4-related aortitis to mimic both intramural hematoma and acute coronary syndromes; illustrating a critical diagnostic blind spot in acute cardiovascular care; particularly when imaging findings; clinical presentation; and intraoperative observations are discordant.
Keywords:
aortitis
chest pain
NSTEMI
acute aortic syndrome
acute coronary sydrome
igG4
igG4 - related disease
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