Nephritic Syndromes Flashcards by Joe Bloggs

What are the characteristics of nephritic syndrome?

Limited proteinuria (<3.5g/day)
Oliguria and azotemia
Salt retention w. periorbital edema and hypertension
RBC casts and dysmorphic RBCs in urine

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What will a biopsy show in nephritic syndrome?

Hypercellular, inflamed glomeruli

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What does C5a attract?

Neutrophils

- Mediate damage in nephritic syndromes

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What does PSGN arise after?

Group A (B hemolytic) strep infection

Of skin
Or pharynx

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What do nephritogenic strains of strep carry as a virulence factor?

M protein

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How long after infection does PSGN usually present?

2 - 3 weeks

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What do patients with PSGN usually present with?

Hematuria (cola-coloured urine)
Oliguria
Hypertension
Periorbital edema
Usually seen in children, but may occur in adults

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What kind of hypersensitivity reaction is PSGN?

Type 3 - immune complex deposition

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What happens to complement levels in PSGN?

Decreased (esp C3)

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What can be seen on histology in PSGN?

LM - Glomeruli enlarged and hypercellular
IF - starry sky granular appearance lumpy bumpy due to IgM, IgG and C3 deposition along BM and mesangium
EM - Subepithelial IC humps

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What is the treatment for PSGN?

Supportive

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What can PSGN progress to in adults?

Rapidly Progressive (crescentic) Glomerulonephritis (RPGN)

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How quickly can RPGN progress to renal failure?

days to weeks

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What is Rapidly progressive (crescentric) GN characterized by?

Crescents in Bowman space

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What are the crescents in RPGN comprised of?

Fibrin and plasma proteins (C3b) with glomerular parietal cells, monocytes, macrophages

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Why is immunofluoresence often required in RP (crescentric) GN?

To resolve etiology

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What will Goodpasture syndrome show on IF

Linear IF

- Antibodies to GBM and alveolar BM this creates a sharp line on IF

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What type of hypersensitivity reaction is Goodpasture syndrome?

Type II

What are classic signs of a patient with RP (crescentric) GN secondary to Goodpasture’s?

Young adult male presents with hemoptysis and hematuria

What is Goodpastures RPGN treated with?

Plasmapheresis

What would cause a negative or Pauci-immune (no Ig/C3 deposition) IF finding in a aptient with RPGN?

Granulomatosis w. polyangitis (aka Churg-Strauss syndrome)
Eosinophilic granulomatosis w. polyangiitis
Microscopic polyangiitis

What is the most common cause of death in SLE?

Renal failure

RP(crescentic)GN
> granular IF, Diffuse proliferative

What cause would Granular IF findings in RP(crescentric)GN be due to?

PSGN

- Diffuse Proliferative (often due to SLE)

Negative/Pauci-immune IF in RPGN with PR3-ANCA/c-ANCA is caused by what?

Granulomatosis with polyangiitis

Negative/Pauci-immune IF in RPGN with MPO-ANCA/p-ANCA is caused by what?

- Eosinophilic granulomatosis w. polyangiitis OR | - Microscopic polyangiitis

What will Diffuse Proliferative GN show on Light Microscopy?

Wire looping of capillaries

What will immunofluoresence show in diffuse proliferative GN?

Granular

What will Electron Microscopy show in Diffuse Proliferative GN?

Subendothelial, sometimes subepithelial or intramembranous IgG-based Ics often w. C3 deposition

What will IgA nephropathy (Berger disease) present with?

Episodic hematuria that usually occur concurrently w. resp or GI tract infections (IgA is secreted by mucosal linings) - Renal pathology of IgA vasculitis

What will histology show in IgA nephropathy (Berger disease)?

LM - Mesangial proliferation IF - IgA-based IC deposits in mesangium EM - Mesangial IC deposition

What is Alport syndrome due to a mutation in?

Type IV collagen

How is Alport Syndrome inherited?

X-linked dominant

What happens to the glomerular BM in Alport syndrome?

Irregular thinning and thickening and splitting of GBM

What other issues are associated with Alport syndrome (type IV collagen mutation)?

- Eye problems (e.g retinopathy, anterior lenticonus) - GN - Sensorineural deafness Can't see, pee or hear a bee!

What will EM show in Alport Syndrome?

"Basket-weave" appearance due to irregular thickening of GBM

How can Membrano-proliferative GN be subdivided?

Type I and II

What can type I Membrano-proliferative GN be secondary to?

Hep B or C infection | - May also be idiopathic

What will be seen on histology in type 1 membrano-proliferative GN?

Subendothelial IC deposits w. granular IF

What will be seen on histology in type 2 membrano-proliferative GN?

Intramembranous deposits, also called dense deposit disease

What is seen on both types of Membrano-proliferative GN?

Mesangial ingrowth - GBM splitting - Tram-track on HandE and PAS stains

What autoantibody is type 2 Membrano-proliferative GN associated with?

C3 nephritic factor (IgG autoantibody that stabilises C3 convertase -> persistent complement activation -> decreased C3 levels

What condition may be seen with Churg-Strauss and not microscopic polyangitis?

Asthma

What is the most common cause of nephropathy worldwide?

IgA nephropathy

What nephritic syndromes often co-present with nephrotic?

- Membrano-proliferative GN | - Diffuse Proliferative GN (crescentic, rapidly progressive w. granular IF)