Nephritic syndrome 12/21 Flashcards

1
Q

LM - enlarged and hypercellular glomeruli, IF - granular, EM - subepitheliam humps. What disease?

A

Acute PSG.

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2
Q

Pathoma. Nephritic syndrome. glomerular disorders characterized by …. 2

A

glomerular inflammation and bleeding

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3
Q

Nephritic syndrome. 4 points in pathoma

A

Limited proteinuria < 3,5g/day
Oliguria and azotemia
Salt retention with periorbital edema and hypertension
RBC casts and dysmorphic RBCs in urine

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4
Q

Pathoma. Biopsy nephritic

A

hypercellular, inflamed glomeruli

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5
Q

Pathoma. Nephritic. What molecules deposited and what is activated

A

Immune complex deposition activates complement;
C5a attracts neutrophils, which mediate damage.

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6
Q

In acute PSG what is deposits in EM?

A

Subepithelial.

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7
Q

What organs infection cause Acute PSG?

A

Pharynx and skin.

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8
Q

What are 2 serologican changes in acute PSG?

A

Increased ASO, decreased C3.

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9
Q

What 3 materials form deposits in acute PSG? Where are those deposits?

A

IgG, IgM, C3. Deposits are along GBM and mesangium.

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10
Q

When is seen acute PSG after A streptococcal infection?

A

2-4 weeks after infection.

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11
Q

Crescents consist of ……

A

Fibrin, macrophages, monocytes, glomerulal parietal cells, plasma proteins (C3b)

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12
Q

Rapidly progressive glomerulonephritis: Linear IF. What is mechanism?

A

Antibodies againts GBM and alveolar basement membrane, e.i. IV type collagen.

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13
Q

Rapidly progressive glomerulonephritis: Negative IF. What is mechanism?

A

In disease there are no Ig/C3 depositions.

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14
Q

Rapidly progressive glomerulonephritis: Granular IF. What is mechanism?

A

Immune depositions. IgG, C3, in acute PSG also IgM.

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15
Q

What is the type of hypersinsitivity in Goodpasture syndrome?

A

II type.

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16
Q

What is the treatment of Goodpasture syndrome?

A

Plasmapheresis.

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17
Q

What clinical appearance is in Goodpasture syndrome?

A

Hematuria and hemoptysis.

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18
Q

What ANCA is in Wegener granulomatosis?

A

c-ANCA/PR3-ANCA

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19
Q

What ANCA is in Microscopic polyangiitis?

A

p-ANCA/MP-ANCA

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20
Q

What means Pauci immune?

A

No Ig/C3 depositions (negative IF).

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21
Q

In what diesease are negative IF?

A

Wegener, microscopic polyangiitis, Churg-Strauss.

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22
Q

What ANCA is in Churg-Strauss?

A

p-ANCA/MP-ANCA

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23
Q

How to distinguish Churg-Strauss and Microscopic polyangiitis?

A

In Churg-Strauss granulomatous inflammation, eosinophilia, asthma.

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24
Q

How to distinguish Wegener and Microscopic polyangiitis?

A

In Wegener upper respiratory infections: sinusitis, rhinitis. Cough, nose deformation. Affected lungs, kidney, skin. Polyangiitis. Necrozing granulomatous inflammation. c-ANCA.

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25
Q

What type of inflammation is in Wegener and Churg-Strauss?

A

Necrozing granulomatous infalmmation.

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26
Q

Most often caused kidney disease in SLE?

A

Diffusive proliferative glomerulonephritis.

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27
Q

What 2 diseases can present as nephritic-nephrotic?

A

Diffusive and membrano proliferative glomerulonephritis.

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28
Q

LM - wire looping of capillaries, IF - granular, EM - subendothelial depositions (sometimes intramembranous). What disease? Deposits are consisted of ……..

A

Diffusive proliferative glomerulnephritis. IgG based often with C3

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29
Q

What diesease presents with wire looping capillaries?

A

Diffusive proliferative glomerulonephritis.

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30
Q

Where are deposits in diffusive proliferative glomerulonephritis?

A

Subendothelial granular.

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31
Q

Most common nephropathy worldwide?

A

IgA nephropathy (Berger disease).

32
Q

How often apears hematuria in Berger disease?

A

Episodic.

33
Q

What diseases appers concurrently with Berger disease?

A

Respiratory and GI infections.

34
Q

What Ig are in Berger disease?

A

IgA

35
Q

What Ig vasculitis is in Berger disease?

A

IgA vasculitis (Henoch - Schonlein purpura).

36
Q

LM - mesangial proliferation, IF - mesangium deposits, EM - mesangial deposits. What disease?

A

IgA nephropathy.

37
Q

In what disease mesangium is the main affected tissue?

A

IgA nephropathy.

38
Q

Why Berger disease is associated with 2 other mucosal infections?

A

In Berger - IgA. In mucosal infections - increased IgA secretion.

39
Q

What gene is affected in Alport syndrome?

A

COL4A5

40
Q

What structure is affected by mutation in particular gene in Alport syndrome?

A

IV collagen, which is founded in basal membranes.

41
Q

,,Can’t see, can’t pee, can’t hear a bee” - what disease?

A

Alport syndrome.

42
Q

What sex chromosome is related with Alport syndrome?

A

X

43
Q

EM - ,,basket weave”. What disease?

A

Alport syndrome.

44
Q

What changes are in GBM in Alport syndrome?

A

Thinning and splitting of GBM,

45
Q

Clinical appearance in Alport syndrome?

A

Eye problems - ocular disturbance (rethinopathy, lens dislocation), glomerunephritis, isolated hematuria, sensory hearing loss.

46
Q

What is affected in Membranoproliferative GN?

A

Mesangium and GBM.

47
Q

EM - Subendothelial, IF - granular. + mesangial IC. What disease?

A

Membranoproliferative GN

48
Q

Membranoproliferative GN type I is associated with ……….

A

HBV, HCV.

49
Q

Membranoproliferative GN type II is associated with ……….

A

C3 nephritic factor (IgG autoantibody).

50
Q

What is dense deposit disease?

A

Membranoproliferative GN type II.

51
Q

Mechanism in Membranoproliferative GN type II.

A

Increased C3 nephritic factor –> stabilized C3 convertase –> persistent complement activation –> decreased C3).

52
Q

In what disease there is persistent complement activation? What structure is associated?

A

In membranoproliferative GN type II. C3 nephritic factor stabilizes C3 convertase.

53
Q

Intramembranous deposits presents in …….. (2).

A

Membranoproliferative GN type II and may present in Diffusive proliferative GN.

54
Q

Mesangial ingrowth –> GBM splitting –> tram track. What disease?

A

Membranoproliferative GN type I and II.

55
Q

GBM splitting in Membranoproliferative GN is caused by ………

A

Mesangial ingrowth.

56
Q

What stains are needed to indicate tram track?

A

PAS or silver.

57
Q

What is tram track?

A

Doubling of GBM.

58
Q

PSGN. IF - due to what depositions?

A

IgG, IgM, C3 - depositions along GBD -> starry sky

59
Q

PSGN. Where are depositions?

A

Subepithelial deposits or ,,humps” along the GBM

60
Q

PSGN. Clinical?

A

Hematuria (coca-cola urine)
Salt retention -> periorbital edema and hypertention
Oliguria
Malaise

61
Q

PSGN - urine?

A

Dysmorphic RBCs, proteins (<3,5g/d), +/- RBC casts

62
Q

UW. PSGN. What lab is decreased?

A

C3 and may be C4

63
Q

UW. PSGN. What patway is activated in complement?

A

alternative –> therefore dec. C3.

64
Q

UW. PSGN. Wat titers are increased?

A

Anti-DNAse and AHase (anti-hyaluronidase)

65
Q

UW. PSGN. Wat titers are increased due to preceeding pharingitis?

A

ASO and anti-NAD (anti-nicotinamice-adenine-dinucleotidase)

66
Q

UW. PSGN. Total complement?

A

Decreased.

67
Q

UW. PSGN. Anemia?

A

Not seen

68
Q

UW. PSGN. What prognosis?

A

in children - excellent (95 proc.)
Age is most important poor prognostic factor (conversely).
Increased age –> poor prognosis.
Progress to RPGN -> end stage renal failure.

69
Q

UW. complement levels in IgA nephropathy?

A

Normal - likely due to weak complement-fixing activity of IgA copared to IgG and IgM

70
Q

UW. PSGN vs IgA nephropathy.

A

PSGN - granular subepithelial depositions consisted with IgG, IgM, C3, decr complement + symptoms few weeks after infection.

IgA nephropathy - IgA depositions in mesangium, normal complement, occurs feww days after mucosal infection, and resolves after several days

71
Q

UW. IgA nephropathy biopsy/IF?

A

Mesangial hypercellularity with mesangial IgA deposits

72
Q

UW. IgA nephropathy with extrarenal symptoms –> what disease?

A

Extrarenal:
GI symptoms (abdominal pain) and bleeding, arthralgias, purpuric skin lesions (papable purpura on buttocks and legs)

73
Q

IgA urinalysis?

A

hematuria
+ since its nephritic -> minimal protein

74
Q

UW. how is called hematuria that occurs in IgA after infection?

A

synpharyngic hematuria

75
Q

the most common cause/mechanism of nephritic syndrome?

A

immune complex depositions

76
Q

Alport abbreviation?

A

cant see, cant pee, cant hear a bee