Abstract: TH-PO355
A Case of Nephrotic Syndrome in an Individual With Severe Autosomal Dominant Polycystic Kidney Disease
Session Information
- Genetic Diseases of the Kidneys: Cystic - I
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Genetic Diseases of the Kidneys
- 1101 Genetic Diseases of the Kidneys: Cystic
Authors
- Salcedo Betancourt, Juan David, The University of Texas Southwestern Medical Center, Dallas, Texas, United States
- Shastri, Shani, The University of Texas Southwestern Medical Center, Dallas, Texas, United States
- Pillai, Anil K., The University of Texas Southwestern Medical Center, Dallas, Texas, United States
- Lakhia, Ronak, The University of Texas Southwestern Medical Center, Dallas, Texas, United States
Introduction
Individuals with ADPKD often have sub-nephrotic range proteinuria. However, nephrotic range proteinuria with symptoms of nephrotic syndrome is rarely reported and presents a diagnostic challenge.
Case Description
A 36-year-old Indian woman with ADPKD diagnosed by imaging and genetic testing 12 years prior to evaluation presented with new-onset edema of hands, legs, and face and worsening hypertension. 24-hour urine collection revealed proteinuria of 6.7g. Serum creatinine remained at baseline. Comprehensive serological workup was negative. Additional history revealed that she was taking Ayurvedic supplements for several months prior to the onset of symptoms. Review of CT scan with interventional radiology identified one small area of preserved renal parenchyma as a potential biopsy target. US-guided kidney biopsy identified membranous nephropathy (MN). Serum and tissue anti-phospholipase A2 receptor antibodies were negative. Age-appropriate malignancy screening was negative. Treatment with Angiotensin II receptor blocker (ARB) and discontinuation of herbal supplements resulted in partial remission (UP/Cr 1.4 g/day) after 2 months, improved blood pressure and resolution of edema.
Discussion
Only a handful of ADPKD cases with biopsy-proven MN have been reported. Use of mercury-containing ayurvedic supplements has been implicated with MN. Renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria to exclude coexisting glomerular disease. Though kidney biopsy confers a significantly greater risk of complications compared to a non-cystic kidney, it can often be safely performed by an interventional radiologist well versed with ADPKD. Referral to tertiary care centers with multidisciplinary teams for ADPKD should be considered. This case underlines the importance of renal biopsy in patients with ADPKD with nephrotic-range proteinuria, for accurate diagnosis and appropriate treatment.
Figure 1. A.Non-contrast CT sagittal section displays bilateral enlarged cystic kidneys. B.Renal ultrasound was used to perform kidney biopsy. The left kidney had a small region of non-cystic parenchyma that was biopsied (red arrows).