Nephrotic Syndrome

NEPHROTIC SYNDROME
DR ALPANA RAIZADA
DR SHILPI SRIVASTAVA (PG MEDICINE)
DEFINITION
• Glomerular disease
Characterised by –
• Protenuria (nephrotic range) - ≥3.5 g or urine PCR ≥2g/g (child)
≥3g/g (adult) or urine ACR ≥2.2 or >50 mg/kg/day in a child or
>40 g/m2/hour in a child.
• Edema
• Hypoalbunemia - <3g/dl
• Hyperlipidemia
• Lipiduria
UPDATE
• Child (<12years) - Nephrotic range proteinuria + edema or

hypoalbunemia
• Adult – Nephrotic range proteinuria + edema + hypoalbunemia
• Hyperlipidemia not mandatory for diagnosis.
CAUSES
Most common causes –

• Child – MCD (85-90%) > FSGS > MN > IgA nephropathy
• Adult – FSGS > MN > MCD > IgA nephropathy
• Elderly – MN > Amyloidosis > FSGS > MCD
CLASSIFICATION
• Primary – due to disease of kidney

• Secondary – renal manifestation of systemic illness
PRIMARY CAUSES
• Minimal change disease

• FSFS
• Membranous nephropathies
• Mesangial proliferative glomerulonephritis
• RPGN
SECONDARY CAUSES
• Diabetes nephropathy
• Amyloidosis
• Lupus nephropathy
• Other glomerular deposition diseases
- Light chain deposition disease
- monoclonal plasma cell disorder
PATHOPHYSIOLOGY
Podocyte injury/GBM Liver – increase

injury/endothelium injury albumin and other
protein synthesis
Protenuria
Activate ENaC
Hypoalbunemia
Salt and water

Decreased oncotic RAAS
retension
pressure activation
(decreased
IVF)
Decreased activity Increased hepatic Decreased
of lecithin lipoprotein lipoprotein lipase
cholesterol acyl synthesis
activity
transferase
Hyperlipidemia
(LDL & VLDL)
Decreased HDL and Increased TG

loss of HDL in urine Increased risk of
cardiovascular
mortality
HYPERCOAGUBILITY
Protenuria Hypercholesterolemia Hypoalbunemia
Urine loss of Increased hepatic Decreased

zymogens, co- Platelet synthesis of intavascular
factors and hyperaggregability Factor V and VIII, volume
coagulation fibrinogen
inhibitors
hypercoagubility Hemoconcentration
Circulating
immune
complexes
DIAGNOSIS
• Clinical presentation
Laboratoy workup
• Blood – Serum albumin, lipid profile, serology for infections and
immune abnormalities, rule out secondary causes, kidney
function tests
• Urine – Urine routine examination, urinary sediments, 24 hour
urine protein, urine ACR/ PCR
• Radiological – Ultrasonography
• Pathological – Biopsy
SOME DEFINITIONS
• Complete remission - protein free urine (urine dipstick – trace

or negative, urine PCR ≤200mg/g) on 3 consecutive days.
• Partial remission – urine PCR 200mg/g to 2000mg/g
• Relapse – nephrotic range proteinuria which reappears after a
minimum of 1 month in remission
• Frequent relapsing NS – steroid sensitive NS with 2 or more
relapses in 6 months or more than 4 in one year.
SOME DEFINITIONS
• Steroid dependant NS – responders who relapse while steroid is

being tapered or within 14 days of stopping steroid treatment.
• Initial non responders – no response during the initial 8 weeks of
therapy.
• Late non responders – an initial steroid responder who fails to
respond to 4 weeks treatment in relapse.
• Steroid resistant NS – absence of remission despite therapy for 4
weeks.
MANAGEMENT
• Diet – salt restriction (<2 g/day)

Anticoagulation
Diuretics ( first correct albumin – 0.5g/kg twice daily before giving
diuretics)
Hypolipidemic agents
ACEI/ARBs – for hypertension (target SBP < 120 mm Hg)
• Child NS – Steroid : 60mg/m2 or 2mg/kg/d
Full dose for 6 weeks f/b taper and stop over 6 weeks (total = 12
weeks).
For child with frequent relapsing NS who develop glucocorticoid
related adverse effects and for all children with SDNS,
glucocorticoids sparing agents are recommended.
Coadministration of glucocorticoids is recommended for ≥2 weeks
(4 weeks) following initiation of glucocorticoids sparing agents.
STEROID SPARING AGENTS
• Cyclophosphamide
• Levamisole
• MMF
• Calcineurin inhibitors
• Rituximab
FRNS
• DOC – oral Cyclophosphamide 2mg/kg/day for 12 weeks

( maximum cumulative dose = 168 mg)
Side effects – dose dependant bone marrow suppression, gonadal
toxicity, secondary AML, haemorrhagic cystitis
• Levamisole
• Not responding – Rituximab
SDNS
• DOC – MMF or Rituximab

• MMF – dose = 1200 mg/m2/day divided 2 doses for 12 month
or 2 years
• Rituximab – 375 mg/m2 for 4 doses, 1 week apart
• 2nd line – Calcineurin inhibitors – Cyclosporin = 4-5 mg/kg/day or,
Tacrolimus – 0.1 mg/kg/day in 2 divided doses for 12 months.
FRNS
• Oral Cyclophosphamide > Levamisol

• Not responding - Rituximab
SRNS
• Plan biopsy
• Genetic studies
INDICATION OF BIOPSY IN A CHILD
• SRNS
• Vasculitis, hypocomplementemia, hematuria
• Before starting Calcineurin inhibitors
• Received Calcineurin inhibitors for 2 years and worsening KFT
• SSNS and fails to respond to steroids in subsequent relapses.
INDICATIONS OF GENETIC STUDIES IN A
CHILD
• SRNS
• Syndromic features
• Congenital NS - <1 year child
• Family h/o FSGS
ADULT NS
• Biopsy all patients

• Rule out secondary causes
• Adult onset MCD – 12 weeks steroid, 6 weeks 1 mg/kg/day f/b
taper over 6 weeks. If at end of 12 weeks, no response, steroid
for another 4 weeks. Total full dose steroids for 16 weeks – SRNS
• If response between 6 weeks to 16 weeks, start tapering after 2
weeks of remission and to be stopped by 24 weeks (6 months).
THANK YOU

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NEPHROTIC SYNDROME

• Child (<12years) - Nephrotic range proteinuria + edema or

Most common causes –

• Primary – due to disease of kidney

• Minimal change disease

Podocyte injury/GBM Liver – increase

Salt and water

Decreased HDL and Increased TG

Protenuria Hypercholesterolemia Hypoalbunemia

Urine loss of Increased hepatic Decreased

• Complete remission - protein free urine (urine dipstick – trace

• Steroid dependant NS – responders who relapse while steroid is

• Diet – salt restriction (<2 g/day)

• DOC – oral Cyclophosphamide 2mg/kg/day for 12 weeks

• DOC – MMF or Rituximab

• Oral Cyclophosphamide > Levamisol

• Biopsy all patients

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