NephrOtic Syndrome


NephrOtic Syndrome
  • Massive prOteinuria (> 3.5 g/day, frothy urine),
  • Hyperlipidemia,
  • Fatty casts, Edema.
  • Associated with thromboembolism (hypercoagulable state due to AT III loss in urine) and  risk of infection (loss of immunoglobulins).
Membranous nephropathy Minimal change disease Focal segmental
glomerulosclerosis
Amyloidosis Membranoproliferative
glomerulonephritis
Diabetic glomerulonephropathy SLE
*Seen in Adults

LM
- diffuse capillary and GBM thickening
IF - granular as a result of immune complex deposition. Nephrotic presentation of SLE.
EM—“spike and dome” appearance with subepithelial deposits.

*Most common cause of 1° nephrotic syndrome in Caucasian adults.
Can be idiopathic or associated with:
- Antibody to phospholipase A2 receptor,
- Drugs (e.g., NSAIDs, penicillamine),
- Infections (e.g., HBV, HCV),
-SLE, or
- Solid tumors.

Poor response to steroid therapy and may progress to chronic renal disease.
*Most common in children.

LM
- normal glomeruli (lipid may be seen in PCT cells).
IF (-).
EM - effacement of foot processes.

May be triggered by:
- Recent infection
- Immunization, or
- immune stimulus.

May be associated with Hodgkin lymphoma (e.g., cytokine-mediated damage).

Excellent response to corticosteroids.
LM—segmental sclerosis and hyalinosis A.
IF (-)
EM—effacement of foot process similar to
minimal change disease.

Most common cause of nephrotic syndrome
in African Americans and Hispanics.
Can be idiopathic or associated with:
 HIV infection,
 Sickle cell disease,
 Heroin abuse,
 Massive obesity,
 Interferon treatment, and
 Chronic kidney disease due to congenital absence or surgical removal.

Inconsistent response to
steroid therapy and may progress to chronic
renal disease
LM—Congo red stain shows apple-green
birefringence under polarized light.

Kidney is the most commonly involved organ
(systemic amyloidosis). Associated with
chronic conditions (e.g., multiple myeloma,
TB, rheumatoid arthritis).
Type I—subendothelial immune complex
(IC) deposits with granular   IF: “tram-track” appearance due to GBM splitting caused by
mesangial ingrowth.

Type II—intramembranous IC deposits; “dense
deposits.”

MPGN is a nephritic syndrome that can also
present with nephrotic syndrome.

Type I is associated with HBV, HCV. May also
be idiopathic.

Type II is associated with C3 nephritic factor
(stabilizes C3 convertase  Ž serum C3 levels).
LM—mesangial expansion, GBM thickening, eosinophilic nodular glomerulosclerosis
(Kimmelstiel-Wilson lesion).

Nonenzymatic glycosylation of GBM→ ↑ permeability, thickening.

Nonenzymatic glycosylation of efferent
arterioles GFR mesangial expansion
LM - Wire Loop

IF - C1q deposits everywhere