Abstract: FR-PO739
A Patient with “Albumin-Dependent” Focal Segmental Glomerulosclerosis
Session Information
- Clinical/Diagnostic Renal Pathology and Lab Medicine - II
November 03, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Glomerular
- 1004 Clinical/Diagnostic Renal Pathology and Lab Medicine
Authors
- Duffy, Margaret, The University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Palmer, Matthew, University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Potluri, Vishnu S., The University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Hogan, Jonathan J., The University of Pennsylvania, Philadelphia, Pennsylvania, United States
Background
Idiopathic focal segmental glomerulosclerosis (FSGS) can present with severe volume overload and acute kidney injury (AKI). Here we present such a patient with idiopathic FSGS complicated by multiple episodes of severe AKI and diuretic-resistant volume overload that was only responsive to IV albumin therapy.
Methods
A 54 year-old Jamaican woman developed nephrotic syndrome (SCr 0.86 mg/dL, UProt:Cr 8 g/g, SAlb 1.7 g/dL). A kidney biopsy revealed tip-variant FSGS. She achieved partial remission within four weeks with prednisone 120 mg/d, but then relapsed (UProt:Cr 3.6, SAlb 2.1 g/dL). She was hospitalized with oliguric AKI (Scr 3.89 mg/dL) and volume overload refractory to high-dose IV diuretics and developed candida esophagitis and C. difficile colitis. Steroids were tapered and she was treated with IV albumin (25%, 1 mg/kg q8h), with improvement in her SCr (1.37 mg/dL) and urine output (8L) within 24 hours (see image). Tacrolimus was started and she was discharged.
She was then re-admitted for volume overload, hypoalbuminemia, and AKI that only responded to 25% IV albumin. Tacrolimus levels were undetectably low. We hypothesized that her acute anasarca led to her inability to absorb tacrolimus. We therefore treated her aggressively with 5 sessions of intermittent ultrafiltration, 5 sessions of plasma exchange therapy with albumin replacement, and pulse oral dexamethasone (40 mg/week). Her AKI again resolved and she was discharged on weekly dexamethasone, oral tacrolimus, and biweekly outpatient albumin infusions. With this therapy she achieved complete remission with target tacrolimus trough levels 6-8 µg/L. She was weaned off steroids and IV albumin. She has been in complete remission for five months on tacrolimus monotherapy.
Conclusion
We present a case of idiopathic FSGS with a rare and extreme phenotype of severe volume overload and recurrent AKI. In these cases, IV albumin therapy should be considered, particularly for patients who were previously steroid-responsive and may therefore be responsive to other immunosuppressive agents.