Open AccessAn atypical presentation of Ormond’s disease
Author(s) -
João Carvão,
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Carlota Vida,
Luís Resende,
Francisca Silva,
Gil Silva,
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Publication year - 2022
Publication title -
revista portuguesa de nefrologia e hipertensão
Language(s) - English
Resource type - Journals
eISSN - 2183-1289
pISSN - 0872-0169
DOI - 10.32932/pjnh.2021.12.160
Subject(s) - medicine , retroperitoneal fibrosis , presentation (obstetrics) , nephrectomy , fibrosis , abdominal aorta , rare disease , surgery , kidney , radiological weapon , disease , pathology , aorta
Retroperitoneal fibrosis is a rare condition defined by an overproduction of fibro-inflammatory tissue in the retroperitoneum, usually involving the abdominal aorta, but also managing to invade adjacent structures. We report a case of a 47-year-old female patient with an atypical radiological presentation of retroperitoneal fibrosis, involving predominantly the right kidney. Ultimately, the diagnosis was performed by an immunohistochemical study that identified IgG4+ plasmocytes after kidney nephrectomy, also known as Ormond’s disease. There are no universal guidelines for the treatment of retroperitoneal fibrosis, due to its rarity and lack of randomized controlled trials comparing different therapeutic strategies.