Abstract: TH-PO623
Successful Treatment of Primary FSGS with LDL Apheresis
Session Information
- Membranous Nephropathy, FSGS, and Minimal Change Disease
October 24, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Nayak, Anjali B., Phoenix Children's Hospital, Phoenix, Arizona, United States
- Lozano-Guzman, German Andres, Phoenix Children's Hospital, Phoenix, Arizona, United States
- Dandamudi, Raja, Washington University in St Louis, St Louis, Missouri, United States
- Zaritsky, Joshua, Phoenix Children's Hospital, Phoenix, Arizona, United States
Background
Focal Segmental Glomerulosclerosis (FSGS) remains one of the most common causes of pediatric end stage kidney disease (ESKD). Its treatment remains empiric and is often unsuccessful with significant treatment morbidity. Additionally, recurrence of FSGS is quite common in renal allograft recipients. Therefore we wanted to describe the efficacy and safety of LDL-A in 4 patients with primary FSGS resistant to multi-drug therapy
Methods
Retrospective, single center study. LDL-A was done using the Liposorber LA -15 system, with 12 treatments over 9 weeks. Patients received IV methylprednisolone with the last 6 treatments ( Table 1).
Results
Primary outcomes were measured by either complete remission (CR), or partial remission (PR). CR was a urine protein to creatinine (UPC) of less than 0.5 mg/mg. PR was a UPC between 0.5 mg/mg to 2 mg/mg, and greater than or equal to 50% reduction on proteinuria. Secondary outcomes included improvement in serum albumin. We treated 4 patients with FSGS resistant to multi-drug therapy. All 4 patients responded and went into complete remission. ( Pic 1 ).
Conclusion
All 4 of our patients were able to achieve complete remission of primary FSGS despite a lengthy history of multi-drug resistance. This builds on existing evidence demonstrating the utility of LDL-A in recurrent disease. Although future studies are needed to determine its mechanism of action, LDL-A is a well-tolerated treatment that may become a standard of care in both primary and recurrent FSGS.
Clinical characteristics of patients at baseline
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
| Age at diagnosis( years) | 3 | 3 | 5 | 2 |
| Age at LDL-A treatment | 4.5 | 4.5 | 5.5 | 14 |
| Initial treatment course | Steroids, mycophenolate, tacrolimus and rituximab | Steroids, mycophenolate, tacrolimus and rituximab | Steroids, mycophenolate, tacrolimus and rituximab | Steroids, mycophenolate, tacrolimus and rituximab |
| Post LDL-A course | Went into CR | Went into CR | Went into CR | Went into CR |