A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta-a clinical and diagnostic challenge

Sinead Gormley; Paola Tacuri Bravo; Xavier Kos; Kamal Solanki; Manar Khashram

doi:10.1016/j.jvscit.2022.02.003

A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta-a clinical and diagnostic challenge

J Vasc Surg Cases Innov Tech. 2022 Mar 8;8(2):275-280. doi: 10.1016/j.jvscit.2022.02.003. eCollection 2022 Jun.

Authors

Sinead Gormley¹, Paola Tacuri Bravo², Xavier Kos³, Kamal Solanki⁴, Manar Khashram¹

Affiliations

¹ Department of Vascular and Endovascular Surgery, Waikato Hospital, Hamilton, New Zealand.
² Department of Histology, Waikato Hospital, Hamilton, New Zealand.
³ Department of Interventional Radiology, Waikato Hospital, Hamilton, New Zealand.
⁴ Department of Rheumatology, Waikato Hospital, Hamilton, New Zealand.

Abstract

Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions.

Keywords: Abdominal aorta; Case report; IgG4-related disease; Pancreatitis; Retroperitoneal fibrosis.

Publication types

Case Reports