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

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Flashcards in 05.14 - Nephritic Syndrome (Nichols, Hastings) - PP + Handout Deck (95):
1

2 Podocyte Disorders

Minimal Change Disease, Focal Segmental Glomerulosclerosis

2

3 basic components of Nephritic Sydnrome

Hematuria, Renal Insufficiency, HTN

3

3 GBM Diseases

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

4

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

C3 Nephritic Factor

5

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

Crescents

6

Age of MPGN presentation

older children and younger adults (7-30)

7

Anti-GBM Disease is characterized by

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

8

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

Primary in children, secondary in adults

9

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

Anti-GBM Disease is characterized by

10

Berger's Disease is aka

IgA Nephropathy

11

BM in Alport Syndrome

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

12

Buzz word for Alport Syndrome

Basket Weave

13

C3 deposition on IF, electron-dense deposits on EM

IF and EM of DDD

14

C3 levels in MPGN 1 vs DDD

Low in both

15

C3 Nephritic Factor

80% of DDD cases

16

Characeteristic feature of IgA Nephropathy

Deposition of IgA1 in MM

17

Characteristic double contour resembling tram tracks on silver stain

MPGN

18

Characteristic LM manifestation in glomeruli of RPGN

Crescents in Bowman's space

19

Clinical manifestation of Endothelial Damage

Hematuria

20

Correlation between anti-gbm titers and disease activity

None

21

Crescentic infiltration causes proliferation of

Mononuclear cells and Parietal Epithelial Cells

22

Crescents in RPGN are composed of

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

23

Crucial feature of Type 3 RPGN

Negative IF

24

DDD is differentiated from other forms of GN

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

25

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

Not by immune complexes, by dysregulation of complement system

26

Demographic of Anti-GBM disease

Young white males

27

Demography of IgA Nephropathy

East Asian male children

28

EM of DDD

Ribbons of dense, dark material deposited w/in GBM

29

Endothelial cell injury in glomerular capillaries can lead to

Thrombus formation

30

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

Thrombotic Microangiopathy

31

Heterozygous females in X-Linked Alport Syndrome

May have hematuria and thin BM

32

Histology of Anti-GBM disease

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

33

How are granular deposits in Type 2 RPGN visualized

EM

34

How are linear deposits in Type 1 RPGN visualrized

IF

35

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

Synpharyngitic: 1-2 Days

36

IF and EM of DDD

C3 deposition on IF, electron-dense deposits on EM

37

IgA Nephropathy differs from post-strep GN in being

Synpharyngitic

38

IgA Nephropathy has immune complex deposition in what location

Mesangial location

39

IgA Nephropathy is differentiated from other forms of GN by

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

40

IgA Nephropathy is typcially triggered by

URTI or GI infection

41

IgA Nephropathy results in ___ pattern of injury

Mesangioproliferative

42

Incidence of IgA Nephropathy is increased in patients with

Celiac Disease or Chronic Liver Disease (decreased hepatobiliary clearance)

43

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

Can be either

44

Is RPGN a medical emergency

No, but requires prompt dx and tx

45

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

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

46

Location of MPGN Deposits

Subendothelial

47

Lupus nephritis immune complex deposition tends to occur in what location

Subendothelial

48

Macular deposits in the eyes and/or acquired partial lipodystrophy

DDD

49

Mesangial proliferation in MPGN is likely in response to

Circulating immune complexes

50

Most common MPGN type

Type 1 (80%)

51

Most common primary GN worldwide

IgA Nephropathy

52

MPGN diseases have LM appearance combining

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

53

MPGN has immune complex deposition in what location

Subendothelial

54

Mutation in Alport Syndrome

Alpha5 chain of Type 4 collagen

55

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

MPGN

56

Other notable location of Dense Deposits in DDD

Bruch membrane of eye

57

Pathogenesis of IgA Nephropathy

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

58

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

Goodpasture's Syndrome

59

Plasmapharesis in Type 2 RPGN

not helpful

60

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

Subepithelial

61

Presentation of Anti-GBM

Nephritic syndrome, Heamturia, rapid renal failure

62

Prognosis in DDD

Poor

63

Prognosis of Anti-GBM disease

Poor - Rapid progressive GN

64

Prognosis of Type 1 MPGN

Poor, slow progression to ESRD requiring dialysis or transplant

65

Progression to Fibrous Crescents

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

66

Renal Insuff can be manifested by

Oliguria and/or Azotemia

67

Ribbons of dense dark material within GBM on EM

DDD

68

RPGN can be characterized by

Type of Glomerular deposits

69

RPGN with Granular (immune complex) deposits

Primary renal disease, secondary renal disease

70

RPGN with linear glomerular deposits

Anti-GMB disease, Goodpasture

71

RPGN with no glomerular deposits

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

72

Secondary MPGN can be a complication of

Hep C

73

Single most characteristic feature of Nephritic Syndrome

Hematuria

74

Systemic form of IgA Nephropathy

Henoch-Schonlein Purpura

75

Triad of Alport Syndrome

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

76

Tx of Anti-GBM disease

Plasmapharesis, Steroids, Immunosuppressives

77

Tx of IgA Nephropathy

Steroids, ACEi's, ARB's

78

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

Immune complexes, activaiton of complement by classical pathway

79

Type 2 RPGN can be seen in severe cases of

Post-Strep GN, Lupus Nephritis, and IgA Nephropathy

80

Type 2 RPGN: in addition to crescents, there is

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

81

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

Thin Basement Membrane Disease

82

What are recruited by damaged endothelial cells in MPGN

Monocytes and Macrophages

83

What causes tram track appearance

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

84

What differentiates MPGN 1 from DDD in IF

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

85

What does IF show in Type 1 MPGN

Granular deposits of C3 and IgG

86

What frequently precedes the kidney diesease in Dense Deposit Disease

URTI

87

What frequently precedes the kidney disease in Type 1 MPGN

URTI

88

What glomerular disease is often associated with Hep C infection

MPGN 1

89

What is abundantly increased in MPGN

Mesangial Matrix

90

What is the basket weave

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

91

What type of immune complex deposition is injurious to endothelial cells

Subendothelial

92

Where are IgA complexes primarily deposited in IgA Nephropathy

Mesangium

93

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

Outside, outside, outside

94

Where is new BM formed in MPGN

At the mesangial cell - endothelial cell interface

95

Which complement pathway is dysregulated in DDD

Alternative