Abstract: PO2423
Steroid-Resistant Retroperitoneal Fibrosis in the Elderly
Session Information
- CKD: Qualitative and Quantitative Observational Studies
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: CKD (Non-Dialysis)
- 2102 CKD (Non-Dialysis): Clinical, Outcomes, and Trials
Authors
- Abdallah, Ahmed, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
- Nauman, Awais, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
- Abuhelaiqa, Essa, Hamad Medical Corporation, Doha, Ad Dawhah, Qatar
Introduction
Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic inflammation and fibrosis that involve multiple organs, including the ureters leading to obstructive uropathy. IgG4-related RPF accounts for the majority of cases. We present a case of obstructive uropathy secondary to RPF that is steroids resistant
Case Description
A 71-year-old male known case of atrial fibrillation on warfarin presents with urinary hesitancy and decrease output. No history of fever, weight loss or night sweats. His creatinine was 1.8mg/dL from baseline of 0.9mg/dL. A CT scan of the abdomen revealed a 15x14cm retroperitoneal lesion extending from aortic bifurcation and obstructing both ureters with moderate left-sided hydronephrosis. The patient required double J-stent (DJS) placement with no improvement in creatinine. Workup revealed elevated IgG4 levels (1215mg/L), normal CBC, negative quantiferon test. Chest X-ray, malignancy screening and PET scan were unremarkable. The patient refused the biopsy given the risk of bleeding. He was started on prednisolone 80 mg for one month with no improvement of hydronephrosis. The patient developed hypertension, hyperglycemia, and edema, hence switching to mycophenolate mofetil (MMF) with low-dose prednisone. Repeated CT scan showed a partial reduction in retroperitoneal fibrosis (12x13cm) with mild hydronephrosis
Discussion
Although rare, secondary causes of RPF such as tuberculosis, Castleman disease, Erdheim-Chester disease, lymphoma and bladder carcinoma should be identified. In idiopathic RPF, high-dose glucocorticoids are the first line of treatment. Several studies have shown the effectiveness of low-dose steroids in combination with immunosuppressive drugs like MMF or rituximab. The use of a combination treatment as a first-line treatment is still debatable, however, patients who cannot tolerate high doses of prednisolone and have poor treatment response should be considered for alternative therapy