Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes - preliminary data

Ren Fail. 2022 Dec;44(1):688-692. doi: 10.1080/0886022X.2022.2064303.

Abstract

Introduction: Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes.

Patients and methods: From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males).

Results: Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p < 0.001); they also had higher serum cholesterol (p < 0.01). Hypertension, diabetes, hyperlipidemia and nicotinism were significantly more prevalent in vascular subtype (all p < 0.001).

Conclusion: Vascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

Keywords: Retroperitoneal fibrosis; acute kidney injury; urorenal type; vascular type.

MeSH terms

  • Female
  • Humans
  • Male
  • Preliminary Data
  • Rare Diseases
  • Retroperitoneal Fibrosis* / diagnosis
  • Retroperitoneal Fibrosis* / therapy