Clinical features, diagnosis, and management of retroperitoneal fibrosis in a university referral hospital and literature review.

Retroperitoneal fibrosis (RPF) is usually idiopathic, although secondary forms also occur. To assess the clinical features, laboratory parameters, imaging findings, and treatment modalities in patients with RPF. Patients diagnosed with RPF at a tertiary care center between 2000 and 2024 were included. For the literature review, PubMed was searched to identify retrospective studies including more than 45 patients with RPF published in the past ten years. Twenty-six patients (11 females) with RPF were identified. The mean age at diagnosis was 59.7 ± 12.4 years. Seventeen patients had idiopathic RPF. In the remaining nine patients, RPF was secondary to: malignancy (19%), radiotherapy (8%), acute pancreatitis (4%), and IgG4-related disease (4%). Fifty percent were current or former smokers. Abdominal pain (65%) and low back pain (42%) were the most frequent symptoms. Elevated acute-phase reactants were observed in 75% of patients, and renal function deterioration in 40%. In the literature review, nine retrospective series with sample sizes > 45 patients were selected. The diagnosis of RPF was made by ultrasound (61%), CT (92%), MRI (8%), and PET/CT (23%). The most frequent pattern of involvement was periaortic-periiliac (50%) and hydronephrosis (50%). Glucocorticoids were the most frequently used medical therapy (69%). The most frequent interventional procedure was ureteral stenting (38%). RPF may be idiopathic or secondary to other etiologies. Abdominal and low back pain are common clinical features. Glucocorticoids remain the most frequently used medical treatment, and ureteral stenting is the most commonly used interventional procedure.