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CRRAB 2 > 2: Renal Path 2 - GN > Flashcards

2: Renal Path 2 - GN Flashcards

1
Q

Gross and micro appearance of cystic renal dysplasia

A

Gross: enlarged, multi-cystic, irregular.
Micro: variably sized cysts lined by flattened epithelium

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2
Q

Etiology of cystic renal dysplasia

A

Sporadic

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3
Q

Inheritance and presentation of adult PCKD

A

AD, bilateral but involving only a portion of the kidney initially, multiple expanding cysts destroy parenchyma

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4
Q

What percent of ADPCKD patients have renal failure at age 75

A

75%

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5
Q

How are cilia involved in adult PCKD?

A

Normally keep cells oriented toward lumen, polycystin muts -> cilia defects -> secretion into inappropriate spaces

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6
Q

Micro appearance of adult PCKD

A

Cysts with variable lining arising from tubules, normal parenchyma between cysts

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7
Q

Clinical presentation of adult PCKD

A

Asymptomatic or pain, hematuria

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8
Q

Adult vs childhood PCKD: gross apperance of kidneys

A

Adult: bag of cysts, lumpy appearance. Children: smooth but enlarged externally, x-sect shows many sm cysts in cortex and medulla

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9
Q

Extra-renal anomalies with adult PCKD (3)

A

Liver cysts (40%), berry aneurysms (cause of death for 4-10%), mitral prolapse (20-25%)

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10
Q

Childhood PCKD inheritance and micro findings

A

AR. Dilation of all collecting tubules. Gross: bilateral issue.

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11
Q

Complications of childhood PCKD

A

Nearly all pts: Liver cysts and bile duct proliferation. Some: congen hepatic fibrosis, periportal fibrosis

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12
Q

What is medullary sponge kidney? Who gets it?

A

Multiple cystic dilations of collecting ducts. Adults.

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13
Q

Symptoms of medullary sponge kidney

A

asymptomatic or hematuria, infection, stone formation. Normal kidney fx.

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14
Q

What is nephronophthisis- uremic medullary cystic disease complex?

A

Cysts in medulla + cortical tubular atrophy + interstitial fibrosis. Onset in childhood, progressive.

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15
Q

Presentation of nephronophthisis- uremic medullary cystic disease complex

A

Polyuria and polydipsia due to the tubular defect

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16
Q

What should you think of for children with polyuria?

A

Tubular defect

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17
Q

What is acquired cystic disease? What is another name for it?

A

Dialysis-associated cystic disease. 7% of dialysis pts will devel renal cell carcinoma w/in the cysts in 10 years

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18
Q

Usual size and location of renal simple cysts

A

1-5 cm usually cortical, clear fluid

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19
Q

GN is ____ mediated

A

immune, hypersensitivity II and III, not usually T-cell (IV)

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20
Q

What are crescents?

A

proliferations of epithelial cells and infiltrating WBCs

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21
Q

Define global and segmental with regards to glomerular pathology

A

Segmental: part of a single glomerulus involved.
Global: Entire single glom involved.

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22
Q

What does trichrome stain highlight? What color?

A

Blue-green basement membrane

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23
Q

How does IF appear with Goodpasture syndrome?

A

Homogeneous, diffuse, ribbon-like (even, not patchy)

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24
Q

Why is there hemoptysis in Goodpasture syndrome?

A

Ab cross reacts with basement membrane of pulmonary alveoli

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25
Q

What happens in membranous nephritis?

A

Autoantibody to M-type phospholipase A2 receptor in kidney -> activation of complement -> damage

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26
Q

How does membranous nephritis appear on IF? EM?

A

IF: granular, interrupted
EM: subepithelial electron dense deposits

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27
Q

What are planted antigens?

A

Non-glomerular origin but plant in kidney where antibodies attack them e.g. DNA, bacterial products such as exotoxin

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28
Q

What is circulating immune complex nephritis? What type of hypersensitivity?

A

Circulating Ag-Ab complexes get trapped in glomerulus. Type III.

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29
Q

Circulating immune complex nephritis IF and EM

A

IF: granular deposits
EM: mesangial, subepithelial or subendothelial deposits

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30
Q

What types of nephritis can result in mesangial deposits?

A

Circ immune complex, Type I MPGN

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31
Q

What types of nephritis can result in subepithelial deposits?

A

Circ immune complex, membranous, Type I MPGN

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32
Q

What types of nephritis can result in subendothelial deposits?

A

Circ immune complex, possible in RPGN, Type I MPGN

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33
Q

What is now known about the rate of progression in glomerular disease?

A

Once GFR reduces to 30-50%, progression to ESRD is at a steady rate regardless of cause

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34
Q

Histological findings of ESRD

A
  1. Focal segmental glomerulosclerosis (FSGS)

2. Tubulointerstitial fibrosis

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35
Q

How does FSGS develop

A

Dec nephron fx -> compens hypertrophy -> hemodynamic change -> segmental sclerosis w cell injury, epithelial loss, accum of proteins

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36
Q

How is FSGS treated?

A

Renin-angiotensin inhibitors slow progression but do not cure

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37
Q

How does tubulointerstitial fibrosis develop?

A

Glom issues -> ischemic tubules downstream of sclerotic gloms + toxic effects of proteinuria -> inc acute & chron inflam -> scarring, fibrosis

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38
Q

Nephritic syndrome is caused by diseases that are ____ and ____ e.g. ____

A

inflammatory and proliferative e.g. post-infectious

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39
Q

What is another name for acute poststreptococcal GN?

A

Post-infectious GN (not always due to strep)

40
Q

What happens in post-strep GN?

A

1-4 weeks post pharyngeal or skin infec w/ certain strains of Gr A beta hemolytic strep -> nephritis

41
Q

What serum findings are present in acute post-strep GN?

A

Low: complement (C3)
High: antistreptolysin O, antiDNase B

42
Q

Post-strep GN histology (3)

A

Large hypercellular glom, prolif of endothelial and mesangial cells, infiltration of neutrophils and monocytes

43
Q

Post-strep GN IF

A

Granular with IgG, IgM, C3 deposits (types not import) in mesangium + along GBM

44
Q

EM of post-strep GN

A

Supepithelial humps “camel” IMPORTANT Also subendo and intramembranous deposits

45
Q

Clinical presentation of post-strep GN

A

Periorbital swelling, malaise, oliguria, “smokey” hematuria, mild HTN, mild proteinuria, red cell casts

46
Q

Prognosis of post-strep GN

A

95% and 60% full recovery of renal fx in kids and adults respectively

47
Q

What is another name for rapidly progressive GN (RPGN)?

A

Crescentic

48
Q

What happens to the glom in RPGN?

A

> 50% of glom have crescents - prolif of parietal epithelial cells of BC + inflam cells

49
Q

Cause of RPGN?

A

No specific entity

50
Q

What is Type I RPGN? IF?

A

Anti-GBM GN. IF: linear ribbon-like deposits in GBM

51
Q

What is Type II RPGN? IF?

A

Immune comp mediated e.g. PSGN, SLE, IgA nephropathy. IF: lumpy-bumpy, granular

52
Q

What is Type III RPGN? IF?

A

Pauci-immune type. Lack of IF staining. Most have P- or C-ANCA

53
Q

In Type III RPGN, will there be signs of vasculitis or glom damage first?

A

Can go either way. May have ANCA pos RPGN without signs of systemic involvement

54
Q

RPGN gross features

A

Large, pale kidneys with petechiae

55
Q

RPGN micro features

A

> 50% of gloms with crescent formation with fibrin strands

56
Q

RPGN EM features

A

Possible ruptures in GBM, with or w/o subepithelial deposits

57
Q

RPGN clinical presentation

A

Nephritic: hematuria, red cell casts, mod proteinuria, HTN, edema

58
Q

RPGN clinical course

A

Progressive over weeks with severe oliguria

59
Q

RPGN treatment

A

Anti-GBM: plasma exchange, steroids, chemo

60
Q

Why is there increased risk of infections + hypercoagulable state in nephrotic syndrome?

A

loss of Ig and complement via proteinuria, loss of anticoagulants esp anti-thrombin III

61
Q

Most common cause of nephrotic syndrome in children

A

Minimal change disease

62
Q

Most common nephrotic syndrome in adults

A

Focal segmental glomerulosclerosis - pts w various renal disease progress to FSGS. #2 is membranous GN

63
Q

Secondary causes/ assocs for membranous GN

A

Drugs (penicillamine, captopril, gold, NSAIDs), malignancy (lung, colon, melanoma), SLE, infections, metabolic (DM, thyroiditis)

64
Q

Pathogenesis of membranous GN

A

IC-mediated, (end or exogenous Ag) often phospholipase A2 receptor of podocytes

65
Q

What is the cause of GBM damage in Membranous GN?

A

Complement-mediated

66
Q

Microscopic findings in membranous GN

A

Normocellular gloms + uniform diffuse thickening of capillary wall (Cheerios look) + Spikes on silver stain (BM between deposits)

67
Q

Later microscopic findings in membranous GN

A

Deposits turn into a very thickened GBM. Late: mesangial sclerosis, glom hyalinization

68
Q

Membranous GN IF

A

Granular deposits along GBM with IgG and C3

69
Q

Membranous GN EM

A

Subepithelial deposits, later spikes of BM grow between deposits, then thickened GBM + foot process effacement

70
Q

Treatment for membranous GN

A

If secondary, treating cause can resolve. Otherwise corticosteroids are +/-

71
Q

Typical course of membranous GN

A

Slow deterioration, chronic proteinuria

72
Q

What is the other term for Minimal Change Disease?

A

Lipoid Nephrosis

73
Q

What is minimal change disease? Peak age?

A

Poss T cell dysfx -> cytokines -> damage to visc epithel cells -> loss of charge barrier/ adhesion defects between epithelial cells

74
Q

MCD associations

A

Atopy (eczema, rhinitis), post resp infec or routine immunization, inc incidence in Hodgkin lymphoma

75
Q

Treatment of MCD?

A

Corticosteroids reverse damage to podocytes

76
Q

Peak age for MCD

A

2-6 years

77
Q

MCD micro findings

A

Normal gloms, prox tubules may be filled with lipid

78
Q

MCD IF

A

No staining. No deposits.

79
Q

EM in MCD

A

Diffuse effacement of podocyte foot processes. No deposits.

80
Q

Clinical course of MCD

A

Massive proteinuria (mostly albumin), no renal failure, often no HTN

81
Q

FSGS associations (4)

A

HIV, heroin addiction, sickle cell disease, morbid obesity

82
Q

Hallmark of FSGS

A

Damage to visceral epithelial cells

83
Q

What genetic abnormalities predispose to FSGS?

A

Muts of proteins that localize to slit diaphragm e.g. nephrin and podocin

84
Q

Some put FSGS on a spectrum with what? Which is more severe?

A

MCD (less severe) – FSGS

85
Q

Micro findings in FSGS

A

Need large biopsy (focal). Collapsed GBM, inc mesangial matrix, hyalinization (Magenta w PAS) +/- foam cells

86
Q

EM findings in FSGS

A

Diffuse effacement of foot processes, focal detachment of epithelial cells from GBM

87
Q

IF in FSGS

A

Mesangial deposits of IgM and C3 in affected area

88
Q

FSGS clinical presentation

A

Nephrotic syndrome, HTN, dec GFR, poor response to corticosteroids

89
Q

Which renal issues recur post transplantation?

A

FSGS

90
Q

HIV nephropathy most common renal complication

A

FSGS

91
Q

HIV nephropathy micro findings

A

Cystically dilated tubules filled w proteinaceous material + inflammation

92
Q

HIV nephropathy EM

A

Tubuloreticular inclusions in endothelial cells (nearly pathognomonic, also in SLE)

93
Q

What is membranoproliferative GN?

A

Prolif of glomerular cells + leukocyte infiltration + GBM changes

94
Q

Membranoproliferative GN clinical presentation

A

Mixed nephrotic - nephritic

95
Q

MPGN associations (4)

A

SLE, HepB, HepC, malignancy

96
Q

Microscopic appearance of MPGN (similar type I & II)

A

Large, hypercellular glom w lobular architecture, thickened GBM w “tram track” of mesangial cell into GBM

97
Q

EM of Type I MPGN

A

Subendothelial deposits +/- supepith and mesangial

CRRAB 2 (12 decks)

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