J Korean Soc Radiol.  2021 Mar;82(2):487-492. 10.3348/jksr.2020.0025.

Unusual Manifestation of Immunoglobulin G4-Related Disease Involving the Retroperitoneum: A Case Report

• Kim B
• Seo JW

• KMID: 2514747
• DOI: http://doi.org/10.3348/jksr.2020.0025

Abstract

Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.

Keyword

Immunoglobulin G4-Related Disease; Retroperitoneal Space; Computed Tomography; X-Ray