J Korean Soc Radiol.  2019 May;80(3):537-542. 10.3348/jksr.2019.80.3.537.

A Rare Case of Granulomatosis with Polyangiitis-Related Periaortitis at the Ascending Aorta

  • 1Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. gshong@amc.seoul.kr
  • 2Department of Radiology, Cheju Halla General Hospital, Jeju, Korea.


Acute aortic syndrome including intramural hematoma is a life-threatening disease encountered in the emergency department with high in-hospital mortality even when a timely diagnosis is made. Therefore, accurate differential diagnosis of acute aortic syndrome and its mimics is important to determine the patient's treatment plan. Until now, a few pathologic diseases mimicking aortic intramural hematoma such as periaortic lymphoma and immunoglobulin G4-elated periaoritis have been reported. Here, we report a rare case of granulomatosis with polyangiitis-related periaortitis misdiagnosed as aortic intramural hematoma presenting to the emergency department with chest pain and similar radiologic findings.

MeSH Terms

Aortic Diseases
Chest Pain
Diagnosis, Differential
Emergency Service, Hospital
Granulomatosis with Polyangiitis
Hospital Mortality
Tomography, Spiral Computed


  • Fig. 1 Granulomatosis with polyangiitis-related periaortitis at the ascending aorta in a 58-year-old man, presenting with chest pain. A. Pre- and postcontrast CT scans (upper and middle panels) show hyperdense aortic wall thickening (arrows) and slight enhancement of the aortic wall. A linear low attenuating line is present within the aortic wall thickening (arrowheads), which indicates a preserved fat plane around the aorta. Postcontrast CT scans (lower panel) show a mass with peripheral enhancement abutting the pleura in the left upper lobe of the lung (arrows). B. 18F-FDG PET/CT shows increased FDG metabolism of the aortic wall (maxSUV = 10.4). 18F-FDG PET/CT shows increased uptake of the lung mass (maxSUV = 12.2). 18F-FDG = 2-F-18-Fluoro-2-Deoxyglucose, maxSUV = maximum standardized uptake value C. Microphotographs of periaortic tissue (upper panel) show lymphoplasmacytic infiltration with necrosis and fibrosis. Fibroblasts can be observed infiltrating the mediastinal fat. These findings suggest non-specific inflammations. There is no evidence of malignancy. Microphotographs of the mass in the left upper lobe (lower panel) show granuloma formations and necrosis. H&E = hematoxylin and eosin stain


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