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Kidney Week

Abstract: PO0325

A Case of AKI with ANCA Vasculitis Associated Retroperitoneal Fibrosis

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms


  • Brown, Leanne, University of Connecticut School of Medicine, Farmington, Connecticut, United States
  • Coombs, Nickolas, University of Connecticut School of Medicine, Farmington, Connecticut, United States
  • Vigneault, Christine B., Greater Hartford Nephrology, Bloomfield, Connecticut, United States

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of multi-system autoimmune small vessel diseases that can give rise to a broad array of clinical signs and symptoms. ANCA-associated retroperitoneal fibrosis (RPF) is an exceptionally rare condition characterized by fibroinflammatory changes in the retroperitoneal space. Most RPF cases are idiopathic, or can be secondary to other medical conditions, the rarest association is with AAV. We present a case of ANCA-associated RPF in a patient with recurrent acute kidney injury (AKI).

Case Description

A 57-year-old male with a history of reactive arthritis presented to the hospital with acute left-lower quadrant abdominal pain and found to have AKI due to obstruction by a soft tissue nodule on the left pelvic sidewall with mild-moderate hydronephrosis. He underwent left ureteral stent placement and his hydronephrosis resolved. Left iliac lymph node pathology was consistent with RPF. He was started on high-dose prednisone with resolution of his AKI. Steroids were tapered after repeat imaging 6 weeks later showed a decrease in the size of the soft-tissue nodule. He subsequently had a series of repeat admissions to the hospital with fevers, hypotension, shortness of breath, and recurrent AKIs. Each time, he was treated with antibiotics and stress-dose steroids with resolution of his symptoms. He was advised to return to the hospital as his symptoms progressed to include hemoptysis and epistaxis. He was found to have proteinase-3 antibodies (PR-3) consistent with granulomatosis with polyangiitis and was started on rituximab.


RPF in association with AAV is an extremely rare condition with very few cases documented in the literature. RPF is an uncommon collagen vascular disease characterized by inflammation of the retroperitoneal space which can produce obstruction notably affecting the abdominal aorta, iliac arteries and ureters. Mostly idiopathic, RPF can be associated with vasculitis, medications, infection, and neoplasms. As highlighted in this case, AAV should be considered with a recently diagnosed RPF and recurrent AKI. A hallmark is improvement of symptoms with steroids. This is important with reference to patient outcomes as delayed treatment, especially when renal disease is present, portends a higher risk of end stage renal disease and early mortality.