Nephritic Syndrome

Question 1

What are the three components of nephritic syndrome?

Answer 1

Hematuria
Renal insufficiency
Hypertension

Acute onset

Question 2

How is renal insufficiency manifested?

Answer 2

Oliguria (decreased urine output) or azotemia

Question 3

What is azotemia?

Answer 3

Increased BUN and creatinine

Question 4

Do patients with nephritic syndrome have proteinuria?

Answer 4

Usually, but only mild

Question 5

What are the diseases that can cause nephritic syndrome?

Answer 5

Membranoproliferative glomerulonephritis
IgA nephropathy
Anti-GBM disease
Alport syndrome

Question 6

Fast facts for membranoproliferative glomerulonephritis type I

Answer 6

Subendothelial immune complex deposition usually secondary to hepatitis C that presents as nephritic syndrome + nephrotic syndrome. Rapidly progressive glomerulonephritis.

C3 + Ig immunophenotype.

TRAM TRACK appearance and thickened BM which is HYPERCELLULAR. Podocyte effacement.

Sometimes associated with LCR monoclonal gammopathies.

Question 7

Fast facts for membranoproliferative glomerulonephritis Type II

Answer 7

C3 nephritic factor - autoantibody that binds C3 convertase and prevents its degradation –> constitutive complement activation.

Pres as macular deposits in eye with partial lipodystrophy.

Tram track appearance

Ig (-)! C3+
RIBBONs - dark ribbons in BM!

Question 8

Fast facts for IgA nephropathy

Answer 8

Galactose deficient IgA is recognized by IgG & IgA resulting in immune complex formation in mesangium.

Most common primary glomerulonephritis worldwide.

POST-URI or GI infection and presents 1-2 DAYS after as highly variable, potential signs include hypertension, hematuria, synpharyngitic hematuria.

50% IgA+

Mesangioproliferative and deposits

NEPHRITIC over nephrotic

Question 9

Fast facts for crescentic glomerulonephritis

Answer 9

Anti-GBM disease of parietal cell accumulation squeezes glomerulus and leads to renal failure.

NEPHRITIC - oligouria, hematuria

Assoc w/ Goodpasture’s, Wegener’s, ANCA+

Type I: linear deposits
Type II: Granular
Type III: pauci-immune - negative immuno

Type III looks like FLAMES

Characteristic crescentic shaped deposit

Question 10

Alport syndrome fast facts

Answer 10

Defective a5 Type IV collagen results in failure of BMs to fuse, leading to BASKET WEAVE appearance in EM

X-linked - but heterozygous females get hematuria

SEEK
Skin
Eyes - lens abnormalities
Ears - sensorineural hearing loss
Kidney - ESRD

Question 11

Thin basement membrane disease fast facts

Answer 11

Defective a3,4 Type IV collagen causes 1/2 thickness GBM

Benign if heterozygous

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