Retroperitoneal fibrosis; a single-centre case experience with literature review

Abstract

We present 13 patients with retroperitoneal fibrosis, focusing on clinical features, radiological characteristics, treatments and their outcomes. Retrospective review of the medical records was performed of all retroperitoneal fibrosis patients diagnosed and treated in our department between 2012 and 2017. Twelve patients were male, with a median age of 64 years. Eleven patients presented with abdominal pain or back pain or both. Aetiologies varied from idiopathic to malignancy and vasculitis. Twelve patients had PET scans. These showed ¹⁸F-fluorodeoxyglucose-avid retroperitoneal soft tissue around the abdominal aorta in the vast majority, with five scans also demonstrating localized or generalized uptake by the aorta. In all cases except one, glucocorticoids were applied as the first-line therapy. Further immunosuppressive therapy was required in 10 cases. Our patients were male and older in age compared with the existing literature. PET scans were very helpful in diagnosis of retroperitoneal fibrosis. Rituximab was found to be an effective treatment in six of our patients.