A case with IGG4-related retroperitoneal fibrosis-periaortitis rapidly diagnosed and dramatically responded to steroid treatment

Abstract

Retroperitoneal fibrosis is a rare disease characterized by development of fibro-inflammatory tissue, which surrounds and causes compression of the retroperitoneal structures such as abdominal aorta, iliac vessels, vena cava and ureters1. It’s prevalence was reported as 1.4 /100,000 2. It has been recently shown that it is one of the IgG4-related disorders3. Herein, we report a male patient who admitted to emergency department with acute abdominal pain, diagnosed with IgG4-related retroperitoneal fibrosis-periaortitis in a very short time, and both high baseline serum creatinine level as well as abdominal pain requiring opioids on admission improved with steroids.