title: Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease.
abstract: Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.
authors.affiliation: Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
authors.forename: Giovanni Maria
authors.initials: GM
authors.lastname: Rossi
authors.affiliation: Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
authors.forename: Rossana
authors.initials: R
authors.lastname: Rocco
authors.affiliation: Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Accorsi Buttini
authors.affiliation: Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Marvisi
authors.affiliation: Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy. augusto.vaglio@virgilio.it.
authors.forename: Augusto
authors.initials: A
authors.lastname: Vaglio
mesh_terms: D001327:Autoimmune Diseases / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000009:adverse effects*; D035583:Rare Diseases / Q000453:epidemiology; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D014057:Tomography, X-Ray Computed / Q000379:methods; D014463:Ultrasonography / Q000379:methods
chemical_list: D007074:Immunoglobulin G
keywords: ANCA; Hashimoto; Hydronephrosis; IgG4; Periaortitis; Retroperitoneal fibrosis; Vasculitis