Abstract: PUB615
Case Report: Spontaneous Remission of Dialysis-Dependent Focal Segmental Glomerulosclerosis
Session Information
Category: Trainee Case Report
- 1204 Podocyte Biology
Authors
- Cheng, Jian, Westmead Hospital, Westmead, New South Wales, Australia
- Parnham, Alan, Gold Coast University Hospital, Gold Coast, Queensland, Australia
- Turner, Megan N/a, Sullivan and Nicolaides Pathology, Bowen Hills, Queensland, New South Wales, Australia
Group or Team Name
- Gold Coast Australia
Introduction
It is uncommon for focal segmental glomerulosclerosis (FSGS) to enter spontaneous remission. As far as the authors are aware there are no reports of a patient recovering renal function from this disease after long term dialysis.
Case Description
A 61 year old Caucasian female presented with nephrotic syndrome and acute kidney injury. There was no relevant medical history (including intravenous drug use) or medications. She was hypertensive and 33 pounds above her baseline. Urinalysis demonstrated 100 red blood cells/hpf, a protein to creatinine ratio of 7.5 g/g, albumin 1.8 g/dL. Creatinine increased from 0.68 to 1.45mg/dL. Vasculitic, infective and myeloma screen was normal. Renal biopsy demonstrated tip variant FSGS affecting 3 of 13 biopsied glomeruli, acute tubular injury, and mild interstitial fibrosis and tubular atrophy (IFTA) (figure 1). She was brought into remission by prednisolone 50mg and cyclosporin 100mg twice daily.
8 months later she re-presented with oligoanuric renal failure. Repeat renal biopsy confirmed FSGS, acute tubular injury and mild IFTA. Immunosuppressant therapy failed therefore was tapered and she was established onto maintenance dialysis. Remarkably 11 months on she spontaneously remitted: peritoneal equilibration test revealed a residual creatinine clearance of 20mL/minute. After ceasing dialysis her creatinine nadired at 1.15mg/dL and protein to creatinine ratio of 0.8g/g.
She remained in remission until 7 years later where (now 70 years old) she became nephrotic with a creatinine of 5.94mg/dL. Biopsy reconfirmed FSGS (NOS category) of one tip, one perihilar and one cellular variant, with acute tubular injury and mild IFTA. After treatment with plasma exchange her creatinine has improved to 2.29mg/dL.
Discussion
This case of spontaneous remission in prolonged dialysis dependent FSGS prompts physicians to monitor the progress of all their patients with FSGS – Even those on dialysis!
Figure 1: FSGS. Tip lesion (arrow)