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Renal Physiology > Nephritic Syndrome - Nichols > Flashcards

Nephritic Syndrome - Nichols Flashcards

1
Q

What are the three basic components of nephritic syndrome?

A

Hematuria
Renal Insufficiency
Hypertension

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2
Q

What are the distinct set of symptoms common to all nephritic syndromes?

A

Oliguria
Hematuria
Hypertension

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3
Q

Is nephritic syndrome typically acute or chronic?

A

acute with onset of days, not weeks

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4
Q

In nephritic syndrome, where are the immune complexes frequently deposited?

A

subendothelial locations,

except for post-infectious glomerulonephritis which tends to be subepithelial

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5
Q

What, in a general pathology sense, can immune complex deposition in subendothelium lead to?

A

complement activation which leads to cell damage, which can lead to thrombus formation in glomerular capillaries (a complication of nephritic syndromes)

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6
Q

How does the immune complex deposition lead to leukocyte infiltration?

A

the immune complexes trigger cellular destruction, which releases cytokine and autocoids. This attracts the leukocytes, which enhances inflammation and can cause glomerular capillary rupture, releasing blood into the urinary space

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7
Q

What usually causes post-infectious glomerulonephritis?

A

an initial case of group A beta-hemolytic streptococci infection, 1-6 weeks after infxn

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8
Q

How does pts present with post-strep glom.?

A

proteinuria, edema, azotemia, htn, gross hematuria, with smoky urine, oliguria in some cases

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9
Q

What complement pathway does post-strep glom use/

A

c4 because c3 levels are low. suggesting an alternative pathway

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10
Q

What titers are usually present in post-strep glom/

A

anti-streptolysin 0 titers, used for dx

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11
Q

What does immunoflu show?

A

diffuse granular deposits of IgG and C3 in glomerular capillary walls and mesangium. HAve dome-shaped subepithelial deposits reffered to as HUMPS

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12
Q

How do you treat post-strep glom?

A

mgmt of htn, loop diuretics, and renal replacement therapy when severe

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13
Q

What will you see upon light microscopy of membranoproliferative glomerulonephritis?

A

increased thickening of the GBM with a proliferation of glomerular cells and infiltration of inflamm cells.

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14
Q

Which of the types of membranoproliferative glom. isn’t actually part of the category?

A

type II, more details later

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15
Q

What is the characteristic appearance of the gbm in membranoproliferative cells upon silver staining?

A

tram tracks, because the two gbm membrane (a new one formed in reparative phase)

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16
Q

What percentage of MPGN type I ?

A

80%

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17
Q

What conditions can lead to mpgn?

A

hepatitis c and many others, usually secondary in adults and primary in children

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18
Q

Where is mpgn more common now?

A

south america, africa and middle east

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19
Q

Explain the patho of type 1 mpgn?

A

mediated by immune complexes causing compl activation by classical pathway. Injury phase with influx of leukocytes leading to proliferative phase, with damage to capillary walls from cytokines and proteases. Endothelial cells lose fenestrations and effacement of podocytes occurs.
reparative changes follow with new basement membrane formation and entrapment of immune complexces and etc leading to the tram track appearance along the capillary walls.

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20
Q

What are the clinical presentations of mpgn 1

A

hemature (macro or micro)
proteinuria
usually follows an upper respiratory tract infxn

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21
Q

What is the tx and prognosis of mpgn1?

A

no consensus tx usually leads to esrd

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22
Q

who typically presents with mpgn?

A

older children and younger adults 7-30

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23
Q

What is dense deposit disease aka?

A

type II mpgn

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24
Q

What is the typically person with dense deposit disease?

A

older children and younger adults 7-30

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25
Q

what causes dense deposit disease?

A

NOT immune-complex mediated but dysregulation of complement activation due to congenital mutations

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26
Q

What abs develop in dense deposit disease

A

anti- factor h factor I factor B and c3 convertase

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27
Q

what does immunofluo show in dense deposit disease

A

granual deposits of c3 along gbm and in mesangium. however they are dense and visible, as opposed to mpgn1.

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28
Q

what is the characteristic description of deposits seen in ddd?

A

ribbon like

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29
Q

Where else can you see ddd?

HOw do you distinguish between ddd and type i mpgn?

A

in the bruch membrane of the eye, leading to decreased vision. lipodystrophy with loss of subcu fat in upper half of body. abs to c3 convertase

30
Q

What is IgA nephropathy also known as?

A

Berger’s disease

31
Q

What is the characteristic feature of IgA nephropathy?

A

depositino of polymeric IgA1 in the mesangial matrix.

32
Q

What are things that lead to secondary IgA nephropathy?

A

celiac disease, chronic liver disease who have reduced hepatobiliary clearance, also IBS, psoriasis, dermatitis,

33
Q

what is the typical person with iga neph?

A

children and young males

34
Q

What typically triggers iga nephr?

A

upper respiratory or gi tract infxn

35
Q

what do you see upon light microscopy in iga neph?

A

mesangial widening

36
Q

what does immunofl show in iga neph/

A

mesangial iga deposits with c3 and properdin

37
Q

what is the clinical presentation of iga neph?

A

microscopic hematuria, gross hematuria at times, proteinuria in nephrotic range, acute glomerulonephritis, htn

38
Q

what do you use to treat iga neph?

A

steroids, acei, arb

39
Q

what is considered the systemic form of iga nephropathy?

A

henoch-schonlein purpura

40
Q

how often do people with iga “relapse”

A

every few months

41
Q

What type of collagen is targeted in Anti-GBM disease?

A

alpha3, compared to alpha v in alport syndrome

42
Q

Where is the expression of the antigen that is targeted in Anti-GBM limited to?

A

the non-collagenous domain of the alpha3 chain of collagen type IV is only expressed in the pulmonary alveolar basement membrane and the gbm

43
Q

What can trigger the formation of the autoantibodies for alpha3 collagen?

A

exposure to hydrocarbons, drugs, viruses tumor or (most commonly) smoking

44
Q

what is the typically person with anti-gbm?

A

young white males

45
Q

What can be seen in histology of anti-gbm disease?

A

crescentic necrotizing gn with linear deposits of IgG along gbm, but not visible by electron microscopy

46
Q

what do pts with anti-gbm prseent with?

A

nephritic syndrome, prominent hematuria and quickly ensuing renal failure, azotemia, arthritis and arthralgia

47
Q

what are signs and symptoms of anti-gbm that involve pulmonary?

A

dyspnea, cough hemoptysis and crackles, anemia due to pulmonary hemorrhage

48
Q

what is a subset but really just another name for anti-gbm disease?

A

goodpasture’s syndrome

49
Q

how can you treat goodpastures/anti-gbm

A

plasmapheresis and steroids and immunosuppressive therapy

50
Q

Is rapidly progressive glomerulonephritis a disease or something that can result from many different disease?

A

something can result from many different diseases

51
Q

What are the characteristics of rapidly progressive glomn?

A

nephritic syndrome, rapid loss of renal fxn, oliguria and if untreated, death in weeks to months.

52
Q

What is seen histologically in rapidly progressive glmni?

A

the presence of crescents in bowman’s capsule, composed of proliferating parietal epithelial cells, macrophages, fibrin, and areas of necrosis

53
Q

Why is the fact that there is proliferation in the bowman’s capsule significant?

A

to be there, it has spread through the endotherlium, gbm, and through the visceral epithelium

54
Q

is rapidly progressive glmni ab-mediated or cell-mediated/

A

can be both

55
Q

what is the consequence of the crescents?

A

they visibly compress the tuft of capillaries, impairing or obliterating blood flod into the tuft.

56
Q

What are examples of linear rapidly progressive glmni?

A

anti-gbm disease/goodpastures

57
Q

what are examples of granular (immune complexes) rapidly prog glmni?

A

primary or secondary renal disease

58
Q

what are exmaples of rapidly progressie glmni that don’t involve any deposition of immune complexes

A

anca-associated, drug-induced, idiopathic

59
Q

What is significant in immunoflu in type II rapidly prog glmni?

A

lumpy bumpy deposits of IgG in the GBM and mesangium

60
Q

What diseases show Type II rapi progr glmni?

A

post-infxs glomermni

iga nephropathy

61
Q

When do you seen type III pauci rapi prog glmni?

A

in people with ANCA

p-anca with skin, kidneys, others, aka microscopic polyangiitis
c-anca in wegeners granulomatosis, kidneys lung and upper respiratory tract

62
Q

What causes alport syndrome?

A

the result of mutations in genes for one of the alpha chains of the collagen type IV (alpha5).

63
Q

what do mutations in the alpha5 chain of type iv collagen result in

A

a defective heterotrime assembly and defective BMs.

64
Q

where is the collagen heterotrimer in alports syndrome used in the body?

A

lens, cochlea, and glomeruli

65
Q

Pts with alport syndrome have problems with what?

A

nerve deafness, lens disorders and nephritis

66
Q

How does the gbm appear on light microscopy in alport syndrome?

A

thin or attenuated. may show several alternating thing lyaers of lamina rara and lamina densa resembling a basket weave

67
Q

What does the mutation in alpha 5 chain prevent?

A

the normal fusion of basement membranes, resulting in “basket weave” pattern

68
Q

how is alports syndrome passed on

A

x-linked, so males are much more affected than the women with the trait

69
Q

how are males affected by alport syndrome

A

persistent hematuria (compared to mild for women), progressive proteinuria and esrd

70
Q

what is defective in thin basement membrane disease?

A

alpha3 chain or alpha4 chain in type iv collagen causing uniform reduction in gbm to about half or normal thickness, presenting with persistent microscopic hematuria

71
Q

what is the typical pt with alport syndrome

A

5-20 yo

Renal Physiology (26 decks)

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