February 8, 2016 – Retroperitoneal Fibrosis

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Anchor lead: An uncommon condition that used to require surgery can now be managed medically, Elizabeth Tracey reports

Retroperitoneal fibrosis is the tongue-twisting name of an inflammatory condition that ultimately constricts urine flow, resulting in pain and other symptoms.  Paul Scheel, a kidney expert at Johns Hopkins, and colleagues, have developed a way to treat it that doesn’t involve surgery.

Scheel: Retroperitoneal fibrosis is inflammation and fibrosis that occurs around the aorta below the level of the renal vessels. It’s very common for patients to present with weight loss, pain, and a number of other vague symptoms and undergo diagnostic workup for up to two years before a diagnosis is finally reached.   Historically retroperitoneal fibrosis was treated surgically. In our initial regimen we chose prednisone 40 mg to start with 10 mg to taper per month, and we added mycophenolate or CellCept as a steroid sparing agent initially.  What was later discovered was that CellCept has antifibrotic properties.   :35

Scheel says the regimen is gaining attention for other autoimmune conditions where fibrosis results.  At Johns Hopkins, I’m Elizabeth Tracey.