SHM Converge Scientific Abstract ePoster Gallery-Atypical Presentation of Aortic Dissection in the Context of Igg4 Disease

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A 59-year-old man with chronic kidney disease from IgG4-related disease and autoimmune pancreatitis, treated with chronic steroids, presented after masturbation with lightheadedness, bilateral upper-extremity numbness, hypotension, and profound bradycardia. He had a loud systolic murmur, thready pulses, delayed capillary refill, mild lactic elevation, and leukocytosis, but no chest pain and a normal chest X-ray. Because he appeared unstable, he was initially treated for possible infection and given fluids, but his blood pressure did not improve. <br /><br />Further evaluation the next day with transthoracic echocardiography revealed a type A aortic dissection, and CT angiography showed extensive dissection from the aortic root involving major arteries. He was transferred to the coronary care unit, started on an esmolol drip, and underwent successful aortic repair with an uncomplicated postoperative course.<br /><br />This case highlights an atypical presentation of aortic dissection without chest pain, which can delay diagnosis and life-saving treatment. It also emphasizes the association between IgG4-related disease, corticosteroid therapy, and aortopathy. While steroids reduce inflammation in IgG4 disease, they may not prevent vascular complications and may contribute to vascular fragility and hypertension. The authors suggest that patients with IgG4-related disease may benefit from baseline and periodic cardiovascular imaging surveillance, such as echocardiography or CT angiography, to detect aortic disease earlier and improve outcomes.

Asset Subtitle

Aphrodite Megaris

Meta Tag

Author List Aphrodite Megaris, Faye Megaris, Mariam Khandaker, William J. Wallach

Category Clinical Vignettes

Concept Aortic Dissection

Concept IgG4-Related Disease

Concept Aortopathy

Concept Computed Tomography Angiography

Concept Atypical Presentation

Distinguished Non-Finalist

Presenter Organization Icahn School of Medicine at Mount Sinai - New York

Presenting Author Aphrodite Megaris

Track Adult

Keywords

aortic dissection

type A dissection

IgG4-related disease

autoimmune pancreatitis

chronic corticosteroid therapy

bradycardia

hypotension

echocardiography

CT angiography

aortopathy

Aortic Dissection

IgG4-Related Disease

Aortopathy

Computed Tomography Angiography

Atypical Presentation