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

• Hong SH
• Hong GS
• Lee CW
• Kim GH

• KMID: 2454032
• DOI: http://doi.org/10.3348/jksr.2019.80.3.537

Abstract

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

Aorta*
Aortic Diseases
Chest Pain
Diagnosis
Diagnosis, Differential
Emergency Service, Hospital
Granulomatosis with Polyangiitis
Hematoma
Hospital Mortality
Immunoglobulins
Lymphoma
Tomography, Spiral Computed
Vasculitis
Immunoglobulins

Figure

• 

  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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