Introduction:

retroperitoneal fibrosis (RPF) is a rare disease characterized by the formation of inflammatory and fibrous tissue in the retroperitoneal space, around the abdominal aorta and iliac arteries, often including the ureters.

Case report:

we present a 90-year-old male patient with RPF treated with meprednisone, who attended to the emergency room due to a ruptured inflammatory abdominal aortic aneurysm.

Discussion:

in RPFs associated with abdominal aortic aneurism, endovascular or surgical treatment is recommended when the aortic diameter is larger than 5.5 cm or when it is rapidly growing (> 1 mm/month) because the danger of rupture would appear to be the same as atherosclerotic aneurysms. However, there are no guidelines or algorithms to follow the vascular management of patients with PRF and a “non-aneurysmal” aorta in the event of failure steroid treatment.

On the other hand, the risk of arterial remodeling and progressive aortic dilatation during steroids treatment has been described. Although the changes are subclinical, there were reported cases in which the patients received surgical treatment due to the rupture, such as in our patient where a complication occurred despite having a maximum aortic diameter of less than 4 cm.

Recently, a series of 6 patients with RPF non-aneurysmal abdominal aorta, that had not responded properly to medical treatment, received infrarenal abdominal aorta endovascular exclusion.

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