Abstract
Objectives: To compare adult patients’ characteristics suffering from idiopathic retroperitoneal fibrosis between “relapse-free” and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. Methods: We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. Results: Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). Conclusion: No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.
Highlights
Idiopathic retroperitoneal fibrosis (IRF) is a rare inflammatory disease characterized by fibro-inflammatory tissue surrounding vessels, especially the abdominal aorta and its branches, with extension into retroperitoneal space
Our data suggest smoking cessation seems related to the absence of relapse, but no features could predict relapse at diagnosis of IRF
Moriconi et al established that lumbar pain was statistically associated with relapse risk [2]
Summary
Idiopathic retroperitoneal fibrosis (IRF) is a rare inflammatory disease characterized by fibro-inflammatory tissue surrounding vessels, especially the abdominal aorta and its branches, with extension into retroperitoneal space. Diagnosis is based on imaging (mostly computed tomography) showing irregular soft tissue mass surrounding the aorta and/or iliac arteries with possible extension to adjacent structures [1]. Exclusion of conditions responsible for retroperitoneal fibrosis (i.e., trauma/surgery, cancer, infections, autoimmune diseases, Ig-G4 related disease, histiocytosis, radiation therapy, and drugs) is mandatory to assess the diagnosis of IRF [3]. Corticosteroids remain the first-line therapy with surgery to relieve obstruction of ureters in case of acute renal failure [1]. About half of patients experienced relapse or evolution to chronic renal failure during the follow-up [1,2] despite steroids. Moriconi et al identified that smoking, lumbar pain, acute renal failure, and antinuclear antibody positivity at diagnosis were associated with relapse risk [2]. We aimed to compare the characteristics of “relapse-free” and relapsing patients at the time of IRF diagnosis
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
