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Il Giornale Italiano di Radiologia Medica 2018 Gennaio-Febbraio;5(1):141-6

DOI: 10.23736/S2283-8376.17.00017-1

Copyright © 2017 EDIZIONI MINERVA MEDICA

language: Italian

Idiopathic retroperitoneal fibrosis as a manifestation of multi-system fibro-inflammatory disorder (IgG4-related sclerosing disease)

Gloria ADDEO, Ginevra DANTI, Diletta COZZI, Monica M. LANZETTA, Simone AGOSTINI, Vittorio MIELE

Sezione Ospedaliera Dipartimentale Radiodiagnostica di Emergenza Urgenza, Dipartimento dei Servizi, Azienda Ospedaliero-Universitaria Careggi, Firenze, Italia


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Retroperitoneal idiopathic fibrosis is a rare disease characterized by fibrous tissue proliferation in the retroperitoneum that, with the involvement of urethral and large abdominal vessels, can cause serious complications. Retroperitoneal idiopathic fibrosis is considered one of the manifestations of a multisystem fibro-inflammatory disorder characterized by tissue infiltration with a dominance of IgG4-positive plasma cells, as a common pathogenic mechanism. The most commonly associated inflammatory and autoimmune fibrous conditions are sclerosing cholangitis and type 1 autoimmune pancreatitis. Here, we report a case of idiopathic retroperitoneal fibrosis with shrunken right kidney and left hydroureteronephrosis, and the concurrent involvement of the hepatic, biliary, and pancreatic systems in a 56-year-old male patient. Knowledge of causal etiopathogenetic mechanisms and associated manifestations has allowed us to make an accurate diagnosis and better stratify patients according to prognosis.


KEY WORDS: Retroperitoneal fibrosis - Immunoglobulin G - Sclerosing cholangitis - Pancreatitis - X-ray computed tomography

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