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Immunoglobulin G4-related periaortitis and periarteritis: CT findings in 17 patients

Dai Inoue1, Yoh Zen, Hitoshi Abo

  • 1Department of Radiology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa 920-8641, Japan. d-inoue@lake.ocn.ne.jp

Radiology
|August 2, 2011
PubMed

Insights

Computed tomographic (CT) imaging reveals immunoglobulin G4 (IgG4)-related arterial lesions, primarily in the aorta and its branches. These findings show characteristic arterial wall thickening on CT scans.

Area of Science:

  • Vascular Imaging
  • Immunology
  • Radiology

Background:

  • Immunoglobulin G4 (IgG4)-related disease is a systemic fibroinflammatory condition.
  • Vascular involvement in IgG4-related disease is increasingly recognized but less understood.
  • Computed tomographic (CT) imaging plays a crucial role in diagnosing and monitoring IgG4-related disease.

Purpose of the Study:

  • To retrospectively evaluate the computed tomographic (CT) findings of immunoglobulin G4 (IgG4)-related disease affecting the vascular system.
  • To characterize the radiological features of IgG4-related periarterial lesions.
  • To correlate CT findings with histopathological features.

Main Methods:

  • Retrospective analysis of CT findings in 17 patients with IgG4-related periarterial lesions.
  • Detailed examination of lesion location, arterial wall thickening, luminal changes, and enhancement patterns.
  • Radiopathologic correlation using surgically resected specimens.

Main Results:

  • Twenty-two periarterial lesions were identified in 17 patients, predominantly in the aorta and its main branches.
  • CT findings included arterial wall thickening (mean 11 mm), circumscription, luminal dilation, exaggerated atherosclerosis, and homogeneous late-phase enhancement.
  • Histopathology confirmed lymphoplasmacytic infiltration, IgG4-positive plasma cells, and fibrosis in the arterial wall, particularly the adventitia.

Conclusions:

  • IgG4-related arterial lesions primarily involve the aorta and its major branches.
  • Radiological hallmarks on CT include homogeneous arterial wall thickening.
  • These findings correspond to IgG4-related sclerosing inflammation in the adventitia.
Abstract

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