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Background 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 ureters. It’s prevalence was reported as 1.4 /100,000 . It has been recently shown that it is one of the IgG4-related disorders. 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.

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