IM
Focal Segmental Glomerulosclerosis (FSGS)
Types:
- Primary
- Secondary: Associated with HIV, IgA nephropathy, reflux nephropathy, Obesity, Heroin use, sickle cell disease, Interferon ttt, Congenital: Charcot MT
Pathology (biopsy):
- LM: Sclerosis & immune deposits in some glomeruli, not all (so focal) and “parts” of glomeruli affected not the whole (segmental)
- LM: segmental sclerosis and hyalinosis
- IM: C negative. IgM, C1, & C3 deposits inside the sclerotic regions
- EM: effacement of podocyte foot processes
Treatment:
- Steroids: Can be tried but not very good response (Up to 6 months)
- Relapse: immunosuppressives: cyclosporine, tacrolimus
- Resistant: Cyclophosphamide, chlorambucil or azathioprine
- Recur in transplanted kidneys
Outcome: Untreated FSGS has a < 10% chance of spontaneous remission
Image: Hypercellular and hyaline deposits
Pedia
Focal Segmental Glomerulosclerosis
- Age (yr): 2 - 10
- Sex (M:F): 1.3:1
- Nephrotic Syndrome: 90%
- Asymptomatic Proteinuria: 10%
- Hematuria: 60 – 80%
- Hypertension: 20% early
- Rate of Progression to Renal Failure: 10 yrs
- Associated Conditions: None