Nephrotic Syndrome (NS)

Isbandiyah,dr, SpPD
 Topic
• Etiology
• Pathophysiology
• Pathology and clinical feature
• Complications
• Diagnosis and differential diagnosis
• Treatment
Symptoms
and signs
 pathological types
• Minimal Change Glomerulopathy
• mesangial proliferative GN
• Mesangial Capilary Glomerulonephritis
• Membranous Glomerulopathy
• Focal Segmental Glomerulosclerosis
 Complications
• Acute renal failure( ARF)
Hypoalbuminemia Hypovolemia pre-renal
azotemia

• Dyslipidemia
• Infection
• Malnutrition
• Loss of immunoglobulins
• Trombosis
 Treatment
Support care
• Rest in bed; limitation of protein intake(0.8-
1.0g/kg/d); limitation of salt intake (<3g/d)

• Diuretic therapy

• Diminishing proteinuria: ACEI and ARB

 Treatment
Inhibition of inflammation and immune response

• Corticosteroid therapy (onset):

for children: prednisone 60mg/m2/d
for adult: prednisone 1mg/kg/d (<80mg/d)

4-6 weeks later , complete remission of proteinturia

occurs, the dosage then decreased (10% every 1-2
weeks).

• Be careful for the side effects of corticosteroid therapy

 Patterns of response of cordicosteroids

Prognosis:
Primary responder, no relapse (steroid sensitive)

Primary responder with only one relapse in the first 6 mo after an initial
response
Initial steroid response with two or more relapses within 6 mo (frequent
relapse)
Initial steroid-induced remission with relapses during tapering of
corticosteroid, or within 2 wk after their withdrawal (steroid dependent)
Steroid-induced remission, but no response to a subsequent relapse

No response to treatment (steroid resistant)

 Treatment
• Cytotoxic drugs with corticosteroid:
(for steroid dependent or steroid resistant)
Cyclophosphamide (CTX): p.o. or intravenously
Side effects: liver injury, inhibition of bone marrow, etc.

• Cyclosporine
(for those failed responsing to combination of steroid and cytotoxic
drugs)
Dose: 5mg/kg/d, bid, p.o.
Side effects: renal and liver toxic injury, expensive, etc.
 Treatment
• Minimal changes: sensitive to steroids; single
drug; combined with cytotoxic drugs when
resistant or dependent on steroids
• Membranous GN: combine steroid with
cytotoxic drugs or cyclosporin; avoid using
drugs when Scr>354umol/L; for the patients
with risks for progressing, otherwise,
investigate 6 months.
 Treatment
• FSGS: sensitive to steroids in 30-50% of
patients; slow response to therapy; steroids
therapy (onset) for 3-4 months; if not
response until 6 month (resistant), then try
cyclosporine.
• Mesangial proliferative GN: no evidence show
that adults will response to steroids
 Treatment
Treatment for complications
• Infection
• Thrombosis
• ARF（HD; cordicosteroids, diuresis）
• dyslipidemia