Week 9: Glomerular Disease Flashcards

1
Q

What are the Nephrotic Syndromes

A

Diabetic Nephropathy, Amyloidosis, Minimal Change Disease, Focal Segmental Glomerular Sclerosis, Membranous Nephropathy, and Membranoproliferative Glomerulonephritis

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2
Q

What are the characteristics of Nephrotic Syndrome

A

3.5 g protein/day in urine
Decreased Albumin
Edema
Hyperlipidemia

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3
Q

What are the Nephritic Syndromes

A

Alport Syndrome, IgA Nephropathy, Rapid Progressive Glomerulnephritis, Infection-Associated Glomerulonephritis, and Membranoproliferative Glomerulonephritis

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4
Q

What are the characteristics of Nephritic Syndrome

A

Hematuria
Hypertension
Proteinuria
Azotemia

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5
Q

ID - PCT, DCT, Glomerulus, Bowman’s Capsule, Mesangium,

A
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6
Q

ID - L, E, M, FP, P, and arrow

A

L - capillary Lumen
E - endothelium
M - Mesangial Cells
FP - foot processes
P - podocyte
Arrow - basement membrane

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7
Q

ID

A

Podocytes and Foot processes

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8
Q

ID

A

Capillary fenestrae

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9
Q

ID

A

Foot processes and fenestrae

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10
Q

ID

A

Granular pattern (immune complexes)
and
Linear pattern (no immune complexes)

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11
Q

are podocytopathies

A

Minimal Change Disease (MCD) and Focal Segmental Glomerular Sclerosis (FSGS)

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12
Q

most common nephrotic syndrome in kids

A

Minimal Change Disease (MCD)

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13
Q

ID

A

Minimal Change Disease (MCD) - normal glomerulus PAS stain and Negative IF

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14
Q

ID

A

Minimal Change Disease (MCD)

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15
Q

ID

A

Minimal Change Disease (MCD)

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16
Q

Pathogenesis of Minimal Change Disease (MCD)

A

immune mediated cytokine injury of podocytes
secondary to infection, HL, and drugs (NSAIDs)

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17
Q

ID

A

“Focal” Segmental Glomerular Sclerosis

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18
Q

ID

A

Focal “Segmental” Glomerular Sclerosis

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19
Q

Common cause of nephrotic syndrome in adults (particularly Hispanic and African American adults)

A

Focal Segmental Glomerular Sclerosis

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20
Q

Primary/idiopathic
secondary: drugs (interferon), heroin, HIV, sickle cell disease, massive obesity “Collapsing Variant” is associated with HIV and has a poor prognosis

A

Focal Segmental Glomerular Sclerosis (FSGS)

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21
Q

ID

A

Membranous Nephropathy - thickened Basement Membrane with PAS stain

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22
Q

ID

A

Membranous Nephropathy - spikes and domes with Jones/silver stain

23
Q

ID

A

Membranous Nephropathy - immune complexes (blue) and spikes/domes (orange), and immune componenets (mainly IgG)

24
Q

Primary: 75% of cases, commonly due to anti phospholipase A2 receptor antibodies (PLA2R)
Secondary to infection (Hepatitis B or C, HIV, syphilis), drugs (NSAIDS, Penicillamine, Gold), Lupus, or solid tumors

A

Membranous Nephropathy (MNP)

25
Q

ID - cells present

A

neutrophils

26
Q

ID

A

Infection-Associated Glomerulonephritis (IAGN) - neutrophil and C3/IgG immune complexes

27
Q

ID

A

Infection-Associated Glomerulonephritis (IAGN) - immune complex humps

28
Q

Bacterial antigen deposits in BM during infection, then immune response leads to immune complex formation (subendothelial and subepithelial) and inflammation with BM injury, the complexes clear in time but the large subepithelial complexes persist longer

A

Infection-Associated Glomerulonephritis (IAGN)

29
Q

ID

A

Membranoproliferative Glomerulonephritis - tram tracking (double countour basement membrane - looks like donuts)

30
Q

ID

A

Membranoproliferative Glomerulonephritis - subendothelial immune complexes

31
Q

Chronic immune mediated glomerular injury; may be primary/idiopathic, or secondary to: infection (HepB or HepC), drugs, Lupus, malignancy

A

Membranoproliferative Glomerulonephritis

32
Q

What is Dense Deposit Disease (DDD)

A

a C3 subtype of Membranoproliferative Glomerulonephritis

33
Q

ID

A

Rapidly Progressive (Crescentic) Glomerulonephritis

34
Q

Type 1 Rapidly Progressive (Crescentic) Glomerulonephritis

A

Anti GBM Disease (Goodpasture’s Disease if lung involved) with Linear IF

35
Q

Type 2 Rapidly Progressive (Crescentic) Glomerulonephritis

A

Immune Complex Disease with proliferative PEC Granular IF

36
Q

Type 3 Rapidly Progressive (Crescentic) Glomerulonephritis

A

ANCA-Positive Vasculitides with Pauci Immune IF

37
Q

ID

A

Anti Neutrophil Cytoplasmic Antibodies/C-ANCA (cytoplasm staining) and Anti Neutrophil Cytoplasmic Antibodies/P-ANCA (perinuclear staining)

38
Q

Damaged basement membranes are leaky, leads to RBCs, fibrin, and macrophages in bowman’s space with proliferation of parietal epithelial cells (crescents).

A

Rapidly Progressive (Crescentic) Glomerulonephritis

39
Q

ID

A

Lupus Nephritis

40
Q

ID

A

Lupus - full house immunoreaction on IF

41
Q

ID

A

IgA Nephropathy - Mesangial Proliferation PAS Stain and Mesangial IgA deposition

42
Q

Mucosal infection leads to circulation of abnormal IgA (IgA is mucosal secretions) polymers which get deposited in the mesangium, causing immune complex formation and basement membrane injury

A

IgA Nephropathy

43
Q

ID

A

Alport Syndrome - basketweaving (focal thinning of the basement membrane with splitting and lamellation)

44
Q

Mutation of type IV collagen (many different mutations possible) causing defective collagen

A

Alport Syndrome

45
Q

ID

A

Diabetic Nephropathy - Hyaline arteriolosclerosis and nodular sclerosis (Kimmelstiel-Wilson nodules)

46
Q

Systemic non-enzymatic glycation of tissues, particularly vessels, making them leaky, protein deposits in the vessel walls (hyaline arteriosclerosis); efferent arteriole is preferentially affected

A

Diabetic Nephropathy

47
Q

ID

A

Amyloidosis - fibrillary deposits in mesangium

48
Q

ID

A

Amyloidosis - pink material in mesangium and salmon colored with congo red stain. + apple-green birefringence with polarized light. AL Amyloidosis can be caused by kappa light chains in Multiple Myeloma, SM, and MGUS.

49
Q

Non soluble fibrillary aggregates of misfolded proteins (several types); cause pressure induced necrosis in glomeruli

A

Amyloidosis

50
Q

c-Anti Neutrophil Cytoplasmic Antibodies/c-ANCA (cytoplasm staining) are antibodies against what

A

PR3 protein (3 and C are both third)

51
Q

p-Anti Neutrophil Cytoplasmic Antibodies/p-ANCA (perinuclear staining) are antibodies against what

A

MPO

52
Q

a condition related to p-Anti Neutrophil Cytoplasmic Antibodies/C-ANCA (cytoplasm staining)

A

Granulomatosis with Polyangiitis (GPA); Wegener’s Granulomatosis

53
Q

a condition related to p-Anti Neutrophil Cytoplasmic Antibodies/P-ANCA (perinuclear staining)

A

Microscopic Polyangiitis