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Abstract: PO1958

A Case of C-ANCA Associated Retroperitoneal Fibrosis and Periaortitis

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Srinivasa, Vinay, Darling Downs Hospital and Health Service, Toowoomba, Queensland, Australia

Group or Team Name

  • Darling Downs Health Service, QLD Australia

Granulomatosis with Polyangitis (GPA) is a type of small vessel vasculits that has prevalence rate of 25-160 cases per million population, and a incidental rate of 0.4 cases per 100,000 population per year.
Clinical features of the disease invlove the respiratory and renal systems. However, large vessels such as the Aorta and retroperitoneal tissue are rarely involved.

Case Description

We present the case of a middle-aged male who presented with an obstructive nephropathy in which abdominal CT revealed a soft tissue mass encompassing the Aorta and Inferior Vena Cava causing obstruction of the Left ureter. Despite ureteric stenting, serum creatinine failed to improve. Furthermore urinanalysis demonstated an active urinary sediment; hemproteinuria. Serum c-ANCA and PR-3 antigen titres were positive. Renal biopsy was performed and confirmed pauci-immune vasculitis.
Our patient was induced with pulsed intravenous methylprednisolone and cyclophosphamide and as part of his maintainence treatment received prednisolone and oral cyclophosphamide.
On follow up, partial remission has been achieved with his serum creatinine returning to baseline level and proteinuria reduced, though erythrocytes are still evident. Repeat abdominal imaging has revealed a reduction in the size of the soft tissue mass with treatment.


Biopsy proven vasculitis has been shown in patients with retroperitoneal fibrosis.
Few case reports and series have described this association, inferring a pathogenic role of ANCA in the development of retroperitoneal fibrosis.
Moreover it has been suggested that retro peritoneal fibrosis may be an early clinical manifestation of ANCA associated vasculitis.
Consequently, ANCA associated vasculitis should be considered in the differential diagnosis of any patient who has Retroperitoneal fibrosis and an active urinary sediment