nephritic syndrome Flashcards

1
Q

what does the site of the glomerular injury have to do with the presentation of nephritic or nephrotic ?

A

podocyte injury is associated with protein loss only
endothelial and mesangial cells are associated with nephritic syndrome

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2
Q

what are the major causes of the nephritic syndrome ?

A

post streptococcal
IgA nephropathy
diffuse proliferative
Rapidly progressive
Alports syndrome
Memebranoproliferative GN ( could do both )

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3
Q

when does post strep GN happen ?

A

after a group A beta hemolytic strep infection , either presenting as impetigo or pharyngitis

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4
Q

what is the pathophysiology associated with post strep GN ?

A

immune complex deposits on the GBM - which are C3, IgM and IgG
associated with hypocomplementemia

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5
Q

what is the appearance of post strep GN in light microscopy vs in immunofluorescence vs electron microscopy?

A

light - enlarged and hypercellular glomeruli due to inflammation
immunofluorescence - granular , starry night appearance or lumpy bumpy
electron microscopy - subepithelial iC lumps

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6
Q

what is the classic case associated with post strep GN ?

A

child
2-3 weeks after a strep throat infection
develops nephritic syndrome

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7
Q

what is the hallmark of post step GN on electron microscopy ?

A

subepithelial humps

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8
Q

what is the prognosis of post strep GN ?

A

better prognosis in children
worse in adults - can develop RPGN

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9
Q

what is the other name for IgA nephropathy and what is the common presentation ?

A

bergers disease
IgA deposition in the mesangium , caused by viral infections presents 2-3 days after onset of illness
usually a UI or GI illness

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10
Q

what is the underlying problem associated with IgA nephropathy ?

A

an overactive immune system

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11
Q

what is the classic presentation associated with IgA nephropathy ?

A

occurs concorruently with respiratory or GI infections

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12
Q

what is the appearance on light microscopy vs immunofluorescence vs electron microscopy for IgA nephropathy ?

A

light microscopy - mesangial proliferation
immunofluoresence - IgA based IC in the mesangium granular appearance
electron microscopy - mesangial IgA deposition

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13
Q

what is the classic case associated with IgA nephropathy ?

A

recurrent episodes of hematuria since childhood
episodes come after URI or diarrheal illnesses
slowly worsening renal function over time

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14
Q

what is the difference in the onset of illness between IgA nephropathy and post strep GN ?

A

post strep - GN : weeks after infection
IgA GN : days after infection

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15
Q

what ttype of vasculitis is associated with IgA nephropathy ?

A

henoch schonlein purpura

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16
Q

what is the mechanism associated with DPGN ?

A

diffuse proliferative GN
often due to SLE , often presents with other SLE features : fever rash and arthritis

17
Q

what is the most common form of lupus nephritis in SLE patients ?

A

diffuse proliferative GN

18
Q

what is the appearance of diffuse proliferative in light microscopy vs immunoflouresence vs electron microscopy ?

A

light microscopy - wire looping ( capillary loops are thickened)
immuno - granular appearance
electron : subendothelial sometimes subepithelial or intra membranous IgG based IC with C3 depositions ( gives a full house pattern)

19
Q

what is the presentation of diffuse proliferative glomerulonephritis ?

A

mixed clinical presentation
can present with both proteinuria and hematuria

19
Q

what is the other name for rapidly progressive GN ?

A

cresenteric GN

20
Q

what type of HS is post strep GN vs cresenteric GN ?

A

post strep - is a type 3 HS
cresenteric is - is a type 2 HSR

21
Q

what is the presentation of RPGN ?

A

acute deterioration of renal function in the setting of vasculitis
they present with fatigue and anorexia

22
Q

what is seen on light microscopy vs immuno vs electron microscopy in RPGN ?

A

light : crescent moon shape consists of plasma proteins and fibrin
immuno : linear IF due to antibodies in the GBM

23
Q

what are the three different types of RPGN ?

A

type 1 : anti GBM disease linear along the GBM
type 2 : immune complex mediated bumpy or granular
type 3 : pauci immune barley anything there

24
what HS is associated with goodpasteurs syndrome ?
type 2 hypersensitivity
25
what are the targets for the antibodies in type 1 RPGN and what is the associated presentation ?
antibodies against alpha 3 chain of type 4 collagen cough and nephritic syndrome
25
what type of HS reaction is type 2 RPGN ?
type 3 often a progression of post strp GN or lupus nephritis
26
what is the associations with RPGN type 3 ?
ANCA positive ( c-ANCA or p-ANCA) most patients will have vasculitis
27
what are the different vasculitis associated with c Anca and p ANCA ?
c ANCA - wegners granulomatosis p-ANCA - microscopic polyangitis , churg strauss syndrome
28
what do all the RPGNs have in common ?
cresents shown on biopsy
29
what is the other name for alports syndrome and what is the associated patholgy ?
hereditary nephritis , genetic type 4 collagen defect
30
what is the classic triad of alports syndrome ?
hematuria hearing loss ocular disturbances
31
what appears on electron microscopy with alports syndrome ?
basket- weave due to irregular thickening and splitting along the GBM
32
which layer of the Glomerulus is affected by alports syndrome ?
splitting of the lamina densa