Bilateral Hydronephrosis From Retroperitoneal Fibrosis

Retroperitoneal fibrosis (RPF) is a rare condition characterized by fibroinflammatory tissue infiltrating and compressing retroperitoneal structures. While mostly idiopathic (idiopathic retroperitoneal fibrosis or IRF), RPF is frequently associated with certain drugs, infections, and malignancies. It is thought to be immune-mediated because of response to steroids and RPF is commonly seen with other autoimmune diseases, especially IgG4-related disease (IgG4-RD). IRF is also a part of the chronic aortitis syndromes and the presence of aortic aneurysms is another characteristic of this disease. A 63-year old woman presented with left-sided flank pain. Computed tomography (CT) scan showed left hydronephrosis from compression of the ureter by a retroperitoneal mass. A thoracoabdominal aneurysm was also noted. A [F]- fluorodeoxyglucose positron emission tomography (FDG-PET) scan showed hypermetabolism in the mass, with no abnormally increased activity noted elsewhere. Within four months, the mass enlarged to involve the right ureter as well, leading to right hydronephrosis. She required bilateral ureteral stents and aneurysm repair. Biopsy of the mass showed dense fibrosis with a mononuclear cell infiltrate. The histology of the aneurysm specimen showed chronic periaortic inflammation. Laboratory investigations were significant for elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), with no evidence of monoclonal gammopathy. She was referred to the rheumatology clinic to receive steroid treatment for IRF. IRF commonly involves the ureters and is diagnosed on CT scans during a workup for obstructive uropathy. The treatment is high dose steroids, while in resistant cases, other immunosuppressants have been used. The presentation of a patient with IRF can commonly mimic that of urinary calculi and malignancy. While rare, IRF should not be forgotten when evaluating a patient for obstructive uropathy.