Nephritic Syndrome


Nephritic Syndrome
  • an Inflammatory process
  • When it involves glomeruli, it leads to
    • Hematuria and RBC casts in urine.
    • Associated with azotemia, oliguria, hypertension (due to salt retention), and proteinuria (< 3.5 g/day)
Acute poststreptococcal
glomerulonephritis
Rapidly progressive
glomerulonephritis
(RPGN)
Diffuse proliferative
glomerulonephritis
IgA nephropathy
(Berger disease)
Alport syndrome
Most frequently seen in children. Occurs ~2 weeks after group A streptococcal infection of the pharynx or skin.
RBC Cast
Resolves spontaneously.

Presents with:
 Peripheral and periorbital edema,
 Dark urine (cola-colored), and
 Hypertension.

LM
—glomeruli enlarged and hypercellular.
IF—(“starry sky”) granular appearance (“lumpybumpy”) due to IgG, IgM, and C3 deposition along GBM and mesangium.
EM—subepithelial immune complex (IC)
humps


Type III hypersensitivity reaction.

 anti-DNase B titers and  complement levels
LM and IF—crescent-moon shape A. Crescents consist of fibrin and plasma proteins (e.g., C3b) with glomerular parietal cells, monocytes, and macrophages.
Several disease processes may result in this pattern, including:
ƒƒ
Goodpasture syndrome:
Hematuria/hemoptysis.
type II hypersensitivity; antibodies to GBM and alveolar basement membrane Ž linear IF
ƒƒ
Granulomatosis with polyangiitis (Wegener):
PR3-ANCA/c-ANCA.

ƒƒMicroscopic polyangiitis
MPO-ANCA/p-ANCA.

Poor prognosis.
Rapidly deteriorating renal function (days to weeks).

Due to SLE or MPGN.

LM—“wire looping” of capillaries.
EM—subendothelial and sometimes
intramembranous IgG-based ICs often with C3 deposition.
IF—granular

Most common cause of death in SLE. DPGN and MPGN can present as nephrotic syndrome and nephritic syndrome concurrently
Often presents/flares with a URI or acute gastroenteritis.
Episodic hematuria with RBC casts.

LM—mesangial proliferation.
EM—mesangial IC deposits.
IF—IgA-based IC deposits in mesangium.
Seen with Henoch-Schönlein purpura

 

Glomerulonephriti
Deafness &
less commonly, Eye problems

Mutation in type IV collagen Ž thinning
and splitting of the glomerular basemen membrane.

Most commonly X-linked