1. LDL Apheresis in Drug Resistant Nephrotic Syndrome
Rodney D Gilbert

2. Patient JM 14 year old girl, presented 06/07/2015
Severe oedema, hypoalbuminaemia and proteinuria
Diagnosis nephrotic syndrome
No response to steroids

3. At Southampton Cushingoid, oedematous Creat 27 µmol/L Albumin 20 g/L
U Prot/creat 4029 mg/mmol
Total cholesterol 16.3 mmol/L
Triglycerides 16.2 mmol/L
Renal Biopsy: FSGS (collapsing variety)

4. Further treatment 1 Tacrolimus (Blood levels 4.3 – 6.4 µg/L)
Bezafibrate 200 mg bd
Continued twice weekly IV albumin
2 weeks later
Plasma albumin 15 g/L
UPC 3192 mg/mmol
MMF 1 g bd added
2 months later
Albumin18 g/L
UPC 1371 mg/mmol
Cholesterol 14.3 mmol/L
TG 7.7 mmol/L

5. Further treatment 2 Rituximab 1 g IV x 2 doses
Remained severely nephrotic, fortnightly IV albumin
After 8 months:
Albumin 14 g/L
UPC 1575 mg/mmol
Cholesterol 12.5 mmol/L
TG 6.4 mmol/L

6. 09/03/2016
First session on the Liposorber!

7. LDL apheresis

9. LDL apheresis Two sessions per week for 3 weeks
One session per week for 6 weeks
Plasma volumes processed increased to 3,500 ml
Last session: Cholesterol > 5.7 mmol/L
LDL C 1.92 post treatment
Albumin 19 g/L
UPC 1365
NOT IN REMISSION!

11. First UK patient treated with LDL apheresis for nephrotic syndrome
A few case reports (no genetics)
2 small series
11 patients: 5 complete remission, 2 partial remission
44 patients: 11 complete remission, 10 partial remission (No genetics)
A few reports of post Tx recurrence patients successfully treated

12. Keeping the customer stratified!
Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management. Kidney Int April 2017
187 patients with SRNS
WES: Genetic cause in 26.2%
No response to drugs, rapid deterioration, no recurrence
No genetic cause in 73.8%
Some responded to CNI, rituximab or MMF
Slower deterioration
47.7% recurrence in graft

13. Planned LDL Apheresis Study
Adults and children
Patients with SRNS with MCNS or FSGS
Drug resistant
Native kidneys or transplant recurrence
No genetic cause found
Watch this space!