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.

Highlights

  • CASE REPORTA 73-year-old white man had presented to the emergency department with a 3-week history of left flank pain radiating to the groin and associated with dark urine

  • Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum

  • Retroperitoneal fibrosis (RPF) is a rare heterogeneous disease of unknown etiology characterized by inflammation and fibrosis of the retroperitoneum that often presents a diagnostic challenge

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Summary

CASE REPORT

A 73-year-old white man had presented to the emergency department with a 3-week history of left flank pain radiating to the groin and associated with dark urine His background included hypercholesterolemia, chronic obstructive pulmonary disease, and an episode of pancreatitis 20 months previously that had been suspected to be related to statin use (Fig 1). A computed tomography (CT) angiogram showed extensive para-aortic soft tissue changes around the abdominal aorta and common iliac arteries causing left ureteral obstruction and moderate hydronephrosis of the left kidney (Fig 2). At this stage, an inflammatory abdominal aortic aneurysm was strongly suspected, with a differential diagnosis of aortitis vs RPF. He remained asymptomatic, with no signs of relapse at 15 months of follow-up

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