title: Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach.
abstract: Retroperitoneal fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches - especially of idiopathic vs. secondary RPF - can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.
authors.affiliation: Unit of nephrology, university hospital, Parma, Italy.
authors.forename: M L
authors.initials: ML
authors.lastname: Urban
authors.affiliation: Unit of nephrology, university hospital, Parma, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Palmisano
authors.affiliation: Unit of nephrology, university hospital, Parma, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Nicastro
authors.affiliation: Section of pathology and laboratory medicine, department of biomedical, biotechnological and translational sciences, university hospital, Parma, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Corradi
authors.affiliation: Unit of nephrology, university hospital, Parma, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Buzio
authors.affiliation: Unit of nephrology, university hospital, Parma, Italy. Electronic address: augusto.vaglio@virgilio.it.
authors.forename: A
authors.initials: A
authors.lastname: Vaglio
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D019411:Clinical Laboratory Techniques; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D035583:Rare Diseases / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D012185:Retroperitoneal Fibrosis / Q000145:classification* / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy
chemical_list: D007074:Immunoglobulin G
keywords: Idiopathic retroperitoneal fibrosis; IgG4-related disease; Retroperitoneal fibrosis; Secondary retroperitoneal fibrosis