Multimodality imaging review of retroperitoneal fibrosis

Abstract

Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease with idiopathic and secondary causes. Idiopathic disease is more common and is believed to be immune mediated; associations with autoimmune diseases and/or inflammatory disorders such as IgG4 related disease are often present. Common complications include hydronephrosis and venous stenosis and/or thrombosis. Due to its nonspecific clinical presentation, imaging is vital for diagnosis; in addition, imaging may help distinguish idiopathic from secondary causes and can aid in distinguishing early/active disease from chronic/inactive disease. Magnetic resonance imaging is the preferred imaging modality to stage and monitor the disease, though CT and PET/CT imaging may also be of value. While the imaging findings can overlap with other diseases, there are some characteristic findings which can favor RPF. However, a biopsy is needed for a definitive diagnosis. The following article discusses the clinical features, imaging appearances across modalities, associated complications, potential diagnostic pitfalls, and treatment approaches for RPF. The role of advanced imaging techniques, such as diffuse weighted imaging and 18F-FDG PET/MRI, in the evaluation of RPF will also be included.