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Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B\u00a0cell\u2013depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases.<\/jats:p>","DOI":"10.1681\/asn.2015101110","type":"journal-article","created":{"date-parts":[[2016,2,9]],"date-time":"2016-02-09T21:59:13Z","timestamp":1455055153000},"page":"1880-1889","source":"Crossref","is-referenced-by-count":164,"title":["Idiopathic Retroperitoneal Fibrosis"],"prefix":"10.1681","volume":"27","author":[{"given":"Augusto","family":"Vaglio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Maritati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2016,2,9]]},"reference":[{"key":"B1-20231016","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/S0140-6736(06)68035-5","article-title":"Retroperitoneal 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