Introduction: Lymphoplasmacytic thoracic aortitis is still underrecognized cause of thoracic aortic aneurysm.
Case report: We herein report a case of symptomatic inflammatory ascending aortic aneurysm and pericarditis diagnosed on CT scan as acute type A intramural hematoma. Patient underwent emergency supracoronary ascending aorta replacement with a prosthetic graft. Marked inflammatory thickening of aneurysm wall was the intraoperative finding. Histopathologic examination of the excised aortic tissue showed inflammation with lymphoplasmacytic pattern of aortitis.
Conclusion: This case demonstrates that lymphoplasmacytic aortitis may mimic symptoms and radiological findings of intramural hematoma.
Keywords: IgG4 related diseases; acute aortic intramural hematoma; inflammatory aortic aneurysm; lymphoplasmacytic thoracic aortitis.