J Korean Soc Radiol.  2017 Nov;77(5):333-338. 10.3348/jksr.2017.77.5.333.

CT Findings of Immunoglobulin G4 Related Periaortitis and Periarterities: A Case Report

• Park JW
• Kim SS
• Lee YM
• Jeong JO
• Park JH
• Lee JH
• Kim JH

• KMID: 2394048
• DOI: http://doi.org/10.3348/jksr.2017.77.5.333

Abstract

Immunoglobulin G4 (IgG4)-related periaortitis and periarteritis are rare systemic inflammatory and fibrosclerosing diseases, usually involving the aorta and its main branches. We report a pathologically confirmed case of IgG4-related periaortitis involving the thoracoabdominal aorta, which can be confused with intramural hematoma or periaortic lymphoma.

MeSH Terms

Aorta
Aortitis
Hematoma
Immunoglobulin G
Immunoglobulins*
Lymphoma
Immunoglobulin G
Immunoglobulins

Figure

• 

  Fig. 1 A 63-year-old woman with IgG4-related periaortitis involving the thoracoabdominal aorta. A. Abdominal ultrasonography revealed localized eccentric hypoechoic aortic wall thickening (arrows, 11 mm in maximal thickness) at the upper abdominal aorta. The lesion seems to be a relatively preserved intima layer, and the thickening mainly occurs in the adventitia and only partly in the media layer of the aorta. B. On initial CTA, semi-circular eccentric, high-attenuated soft tissue lesion at the thoracoabdominal junction of the aorta (arrows) is seen on pre-enhanced axial image. The attenuation of the internal area is 42 HU. C. On initial post-enhanced axial and coronal CTA images, little homogenous enhancement (47 HU) with a smooth border at the same location (arrows), with thickening mainly in the adventitia and only partly in the media layer of the aorta is seen. The maximal wall thickness and longitudinal length of the lesion are about 11 and 105 mm, respectively. Notably, there is no distinct atherosclerotic change in the affected segment of the aorta, and no streaky infiltration around this lesion. CTA = computed tomography aortography, IgG4 = immunoglobulin G4, HU = Hounsfield unit, SD = standard deviation D. After two months, the aortic wall thickness at the thoracoabdominal junction area markedly increased from 11 to 18 mm. The proximal celiac artery enclosed within the lesion shows diffuse luminal narrowing (left arrow). Another prominent circumferential soft tissue lesion around the right proximal common carotid artery is noted (right arrow). E. Histologic specimens reveal diffuse lymphoplasmacytic cell infiltration and some eosinophils, suggesting chronic fibrosclerosing disorder (left arrow, hematoxylin and eosin stain, × 400). Immunochemical staining of IgG4 revealed elevated IgG4-positive plasma cells in the tissue, confirming IgG4-related periaortitis (right arrow, IgG4 immunohistochemical stain, × 400). IgG4 = immunoglobulin G4

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