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Renal > 05.14 - Nephritic Syndrome (Nichols, Hastings) - PP + Handout > Flashcards

05.14 - Nephritic Syndrome (Nichols, Hastings) - PP + Handout Flashcards

1
Q

2 Podocyte Disorders

A

Minimal Change Disease, Focal Segmental Glomerulosclerosis

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2
Q

3 basic components of Nephritic Sydnrome

A

Hematuria, Renal Insufficiency, HTN

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3
Q

3 GBM Diseases

A

Anti-GBM Disease, Alport’s, Thin BM Disease

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4
Q

Ab’s to ___ occur in about 80% of patients with DDD

A

C3 Nephritic Factor

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5
Q

Accumulation and proliferation of cells outside the glomerular tuft which can result in compression of the tuft

A

Crescents

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6
Q

Age of MPGN presentation

A

older children and younger adults (7-30)

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7
Q

Anti-GBM Disease is characterized by

A

Auto-Ab’s against epitope in non-collagenous domain of alpha 3 type 4 collagen

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8
Q

As a general principal, MPGN is usually ___ in children, and ___ in adults

A

Primary in children, secondary in adults

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9
Q

Auto-Ab’s against epitope in non-collagenous domain of alpha 3 type 4 collagen

A

Anti-GBM Disease is characterized by

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10
Q

Berger’s Disease is aka

A

IgA Nephropathy

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11
Q

BM in Alport Syndrome

A

Abnormally thin, with splintering of lamina densa causing basket weave appearance

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12
Q

Buzz word for Alport Syndrome

A

Basket Weave

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13
Q

C3 deposition on IF, electron-dense deposits on EM

A

IF and EM of DDD

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14
Q

C3 levels in MPGN 1 vs DDD

A

Low in both

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15
Q

C3 Nephritic Factor

A

80% of DDD cases

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16
Q

Characeteristic feature of IgA Nephropathy

A

Deposition of IgA1 in MM

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17
Q

Characteristic double contour resembling tram tracks on silver stain

A

MPGN

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18
Q

Characteristic LM manifestation in glomeruli of RPGN

A

Crescents in Bowman’s space

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19
Q

Clinical manifestation of Endothelial Damage

A

Hematuria

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20
Q

Correlation between anti-gbm titers and disease activity

A

None

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21
Q

Crescentic infiltration causes proliferation of

A

Mononuclear cells and Parietal Epithelial Cells

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22
Q

Crescents in RPGN are composed of

A

proliferating parietal epithelial cells, macrophages, fibrin; eventually areas of necrosis

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23
Q

Crucial feature of Type 3 RPGN

A

Negative IF

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24
Q

DDD is differentiated from other forms of GN

A

EM: Ribbons of dense, dark material deposited w/in GBM

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25
Q

DDD is not caused by ___, but instead by dysregulation of ___

A

Not by immune complexes, by dysregulation of complement system

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26
Q

Demographic of Anti-GBM disease

A

Young white males

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27
Q

Demography of IgA Nephropathy

A

East Asian male children

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28
Q

EM of DDD

A

Ribbons of dense, dark material deposited w/in GBM

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29
Q

Endothelial cell injury in glomerular capillaries can lead to

A

Thrombus formation

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30
Q

Glomerular endothelial cell injury and capillary thrombus formation can occur in absence of immune complexes and cause a syndrome called

A

Thrombotic Microangiopathy

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31
Q

Heterozygous females in X-Linked Alport Syndrome

A

May have hematuria and thin BM

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32
Q

Histology of Anti-GBM disease

A

Crescentic necrotizing GN, w/ characteristic linear deposits of IgG along GBM

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33
Q

How are granular deposits in Type 2 RPGN visualized

A

EM

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34
Q

How are linear deposits in Type 1 RPGN visualrized

A

IF

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35
Q

How soon after infection will you see nephritic sediment in IgA Nephropathy

A

Synpharyngitic: 1-2 Days

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36
Q

IF and EM of DDD

A

C3 deposition on IF, electron-dense deposits on EM

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37
Q

IgA Nephropathy differs from post-strep GN in being

A

Synpharyngitic

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38
Q

IgA Nephropathy has immune complex deposition in what location

A

Mesangial location

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39
Q

IgA Nephropathy is differentiated from other forms of GN by

A

IF: characterstic findings of mesangial IgA deposits, often with C3 and properdin

40
Q

IgA Nephropathy is typcially triggered by

A

URTI or GI infection

41
Q

IgA Nephropathy results in ___ pattern of injury

A

Mesangioproliferative

42
Q

Incidence of IgA Nephropathy is increased in patients with

A

Celiac Disease or Chronic Liver Disease (decreased hepatobiliary clearance)

43
Q

Is crescent formation due to antibodies or a cell-mediated process

A

Can be either

44
Q

Is RPGN a medical emergency

A

No, but requires prompt dx and tx

45
Q

It is thought that DDD is a ___ disease, that becomes manifest when ___

A

Two-Hit, infection or autoimmunity gives rise to excess of immune complexes or complement activation

46
Q

Location of MPGN Deposits

A

Subendothelial

47
Q

Lupus nephritis immune complex deposition tends to occur in what location

A

Subendothelial

48
Q

Macular deposits in the eyes and/or acquired partial lipodystrophy

A

DDD

49
Q

Mesangial proliferation in MPGN is likely in response to

A

Circulating immune complexes

50
Q

Most common MPGN type

A

Type 1 (80%)

51
Q

Most common primary GN worldwide

A

IgA Nephropathy

52
Q

MPGN diseases have LM appearance combining

A

Thickened, split GBM w/ a proliferation of glomerular cells and infiltration of inflammatory cells

53
Q

MPGN has immune complex deposition in what location

A

Subendothelial

54
Q

Mutation in Alport Syndrome

A

Alpha5 chain of Type 4 collagen

55
Q

One of the most common diseases to have the nephritic-nephrotic phenotype

A

MPGN

56
Q

Other notable location of Dense Deposits in DDD

A

Bruch membrane of eye

57
Q

Pathogenesis of IgA Nephropathy

A

Aberrant Glycosylation of O-linked glycans in the hinge region of IgA1

58
Q

Patients with Anti-GBM disease who have pulmonary and renal involvement

A

Goodpasture’s Syndrome

59
Q

Plasmapharesis in Type 2 RPGN

A

not helpful

60
Q

Post-infectious GN immune complex deposition tends to be in what location

A

Subepithelial

61
Q

Presentation of Anti-GBM

A

Nephritic syndrome, Heamturia, rapid renal failure

62
Q

Prognosis in DDD

A

Poor

63
Q

Prognosis of Anti-GBM disease

A

Poor - Rapid progressive GN

64
Q

Prognosis of Type 1 MPGN

A

Poor, slow progression to ESRD requiring dialysis or transplant

65
Q

Progression to Fibrous Crescents

A

Segmental proliferative and necrotizing lesions –> Cellular Crescents –> Fibrocellular crescents –> Fibrous Crescents

66
Q

Renal Insuff can be manifested by

A

Oliguria and/or Azotemia

67
Q

Ribbons of dense dark material within GBM on EM

A

DDD

68
Q

RPGN can be characterized by

A

Type of Glomerular deposits

69
Q

RPGN with Granular (immune complex) deposits

A

Primary renal disease, secondary renal disease

70
Q

RPGN with linear glomerular deposits

A

Anti-GMB disease, Goodpasture

71
Q

RPGN with no glomerular deposits

A

Drug-induced, Idiopathic, ANCA-associated (wegener’s or Micrscopic Poly)

72
Q

Secondary MPGN can be a complication of

A

Hep C

73
Q

Single most characteristic feature of Nephritic Syndrome

A

Hematuria

74
Q

Systemic form of IgA Nephropathy

A

Henoch-Schonlein Purpura

75
Q

Triad of Alport Syndrome

A

Nephritis, Nerve Deafness, and Lens disorders (all need defective collagen)

76
Q

Tx of Anti-GBM disease

A

Plasmapharesis, Steroids, Immunosuppressives

77
Q

Tx of IgA Nephropathy

A

Steroids, ACEi’s, ARB’s

78
Q

Type 1 MPGN is mediated by ___, causing activation of _____

A

Immune complexes, activaiton of complement by classical pathway

79
Q

Type 2 RPGN can be seen in severe cases of

A

Post-Strep GN, Lupus Nephritis, and IgA Nephropathy

80
Q

Type 2 RPGN: in addition to crescents, there is

A

segmental necrosis, mesangial cell proliferation, and exudate w/ leukocytes

81
Q

Uniform reduction in GBM to about 1/2 of normal thickness

A

Thin Basement Membrane Disease

82
Q

What are recruited by damaged endothelial cells in MPGN

A

Monocytes and Macrophages

83
Q

What causes tram track appearance

A

Formation of new BM and entrapment of immune complexes, complement factors, cellular elements, and matrix material

84
Q

What differentiates MPGN 1 from DDD in IF

A

MPGN 1 has C3 and IgG, but DDD has just C3

85
Q

What does IF show in Type 1 MPGN

A

Granular deposits of C3 and IgG

86
Q

What frequently precedes the kidney diesease in Dense Deposit Disease

A

URTI

87
Q

What frequently precedes the kidney disease in Type 1 MPGN

A

URTI

88
Q

What glomerular disease is often associated with Hep C infection

A

MPGN 1

89
Q

What is abundantly increased in MPGN

A

Mesangial Matrix

90
Q

What is the basket weave

A

Alport: GBM has several alternating layers of lamina rar and lamina densa

91
Q

What type of immune complex deposition is injurious to endothelial cells

A

Subendothelial

92
Q

Where are IgA complexes primarily deposited in IgA Nephropathy

A

Mesangium

93
Q

Where are immunce complexes formed in MPGN? IgA Nephropathy? Lupus?

A

Outside, outside, outside

94
Q

Where is new BM formed in MPGN

A

At the mesangial cell - endothelial cell interface

95
Q

Which complement pathway is dysregulated in DDD

A

Alternative

Renal (31 decks)

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