Management Of Nephrotic

Syndrome
 Minimal Change Disease
 DIAGNOSIS
 -Kidney biopsy :
Light Microscopy: normal
Immunofluorescece :negative
Electron microscopy: fusion of foot
processed-podocytes
 Treatment
 Prednisone 1mg/kg/d : approx 80 percent of adults
respond with decrease in proteinuria to <3g/d or
resission. Steriods should be tapered over 3 months
and then discontinued
failure to respond FSGS
monitering of urinary protein excretion
Cytotoxic Agents: steriod dependent steriod resistent
frequent relapsers
Cyclophosphamide 2mg/kg/d PO for 8 weeks
Chlorambucil .2mg/kg/d po 8-12 weeks
Cyclosporine 5mg/kg/d for 6-12 months
Progression to CKD-rare
Focal Segmental Glomerulosclerosis
 Diagnosis:
Light microscopy:focal and segmental
sclerosis of glomeruli
Electron microscopy: focal or diffuse foot
process effacement
 Treatment
 Steroids: generally not proven to be effective
trial of prednisone 60mg PO daily 3 months may
reduce proteinuria and slow progression to ESRD
Resistant combination of glucocorticoids and
cytotoxic agents
Cyclosporine 5mg/kg/d PO
Cyclophosphamide 2mg/kg/d PO
Mycophenolate mofetil 1000-3000mg/d
Frequently progresses to CKD and ESRD within 5-
10 yrs of Dx
 Membranous Nephropathy
 Diagnosis:
Light microscopy: thickening of GBM often
with GBM soikes
Immunofluorescence sub-epithelial deposits
of IgG and C3
 Treatment
 Treatment is reserved for
 patients with poor prognostic factors:
age>50 ,male gender ,hypertension,reduced
GFR, proteinuria >10g or marked interstatial
fibrosis on renal biopsy .
 Severe symptomatic nephrotic syndrome.
 High-dose alternate day glucocorticoids in
conjunction with a cytotoxic agent
Chloranbucil 0.2mg/kg/d
Cyclophosphamide 1.5-2.5 mg/kg/dfor 6-12
months
Cyclosporine 3.5mg/kg/d in non responders
Mycophenolate mofetil ,Rituximab , Pentoxifylline
alternative agents.
Approx 1/3rd progress to ESRD remaining remission
or stable or very slow declining renal function
 IgA Nephropathy
 Diagnosis:
Light microscopy: increased mesangial
cellularity and matrix
Immunofluorescence and electron
microscopy: mesengeal depostion of IgA
and C3
 Treatment
 Glucocorticoids in pts with progression
 Omega -3-fatty acid 6 g Po bid including
1.8g eicosapentaenoic acid and 1.2 g
docosahexanoic acid may be beneficial in
preventing deterioration of renal function
 Cyclophosphamide may be used in severe
crescentic disease
 Fewer than 25% of cases progress to ESRD
by 20 yrs
 Membranoproliferative GN
Diagnosis: mesangial proliferation with
collapse of capillary loop alterations of GBM
with subendothelial (Ty I) or
intramembranous(Ty II) electron dense
deposits .
tram tracking ‘mesanial interposition with
GBM’
 Treatment
 Adult idiopathic MPGN treatment has not been
shown to improve disease free survival
Use of steriods in children stablizes disease
HCV associated MPGN improves with successful
antiviral therapy.
Fifty to sixty percent pts with idiopathic MPGN
progress to ESRD by 10-15 yrs.
 Diabetic nephropathy
 Dx
Light Microscopy: glomerular sclerosis and
mesangial expansion with nodular
appearance (kimmelstiel-Wilson nodules)
Electron Microscopy GBM thickening
 Treatment
 Aggressive glucose control and aggressive
BP control with ACEI’s or ARBs or both.
 Most common cause of ESRD
 SLE
 Diagnosis:
 Renal biopsy
mesangial ,membranous,focal or diffuse
proliferative and crescentic GN
Immunofluorescence is + for IgA IgG IgM C3
and C4
Irreversible changes :glomerular sclerosis
tubular atrophy interstatial fibrosis
 Treatment
 Severe Disease: methylprednisolone 500 mg IV
q12hrly for 3 days followed by pral prednisone 0.5-
1mg/kg Po daily should be tapered over 6-8
weeks to lowest dose that controls disease.
 Moderate to severe Induction :mycophenolate
mofetil 1000mgpo tid or cyclophosphamide 0.5-
1.0 g/m2 IV monthly for 6 months Maintenance:
mycophenolate mofetil or quarterly IV
cyclophosphamide for 2 yrs.
 Thankyou
