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

2: Renal Path 2 - GN Flashcards

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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
What happens in membranous nephritis?
Autoantibody to M-type phospholipase A2 receptor in kidney -> activation of complement -> damage
26
How does membranous nephritis appear on IF? EM?
IF: granular, interrupted EM: subepithelial electron dense deposits
27
What are planted antigens?
Non-glomerular origin but plant in kidney where antibodies attack them e.g. DNA, bacterial products such as exotoxin
28
What is circulating immune complex nephritis? What type of hypersensitivity?
Circulating Ag-Ab complexes get trapped in glomerulus. Type III.
29
Circulating immune complex nephritis IF and EM
IF: granular deposits EM: mesangial, subepithelial or subendothelial deposits
30
What types of nephritis can result in mesangial deposits?
Circ immune complex, Type I MPGN
31
What types of nephritis can result in subepithelial deposits?
Circ immune complex, membranous, Type I MPGN
32
What types of nephritis can result in subendothelial deposits?
Circ immune complex, possible in RPGN, Type I MPGN
33
What is now known about the rate of progression in glomerular disease?
Once GFR reduces to 30-50%, progression to ESRD is at a steady rate regardless of cause
34
Histological findings of ESRD
1. Focal segmental glomerulosclerosis (FSGS) | 2. Tubulointerstitial fibrosis
35
How does FSGS develop
Dec nephron fx -> compens hypertrophy -> hemodynamic change -> segmental sclerosis w cell injury, epithelial loss, accum of proteins
36
How is FSGS treated?
Renin-angiotensin inhibitors slow progression but do not cure
37
How does tubulointerstitial fibrosis develop?
Glom issues -> ischemic tubules downstream of sclerotic gloms + toxic effects of proteinuria -> inc acute & chron inflam -> scarring, fibrosis
38
Nephritic syndrome is caused by diseases that are ____ and ____ e.g. ____
inflammatory and proliferative e.g. post-infectious
39
What is another name for acute poststreptococcal GN?
Post-infectious GN (not always due to strep)
40
What happens in post-strep GN?
1-4 weeks post pharyngeal or skin infec w/ certain strains of Gr A beta hemolytic strep -> nephritis
41
What serum findings are present in acute post-strep GN?
Low: complement (C3) High: antistreptolysin O, antiDNase B
42
Post-strep GN histology (3)
Large hypercellular glom, prolif of endothelial and mesangial cells, infiltration of neutrophils and monocytes
43
Post-strep GN IF
Granular with IgG, IgM, C3 deposits (types not import) in mesangium + along GBM
44
EM of post-strep GN
Supepithelial humps "camel" *IMPORTANT* Also subendo and intramembranous deposits
45
Clinical presentation of post-strep GN
Periorbital swelling, malaise, oliguria, "smokey" hematuria, mild HTN, mild proteinuria, red cell casts
46
Prognosis of post-strep GN
95% and 60% full recovery of renal fx in kids and adults respectively
47
What is another name for rapidly progressive GN (RPGN)?
Crescentic
48
What happens to the glom in RPGN?
>50% of glom have crescents - prolif of parietal epithelial cells of BC + inflam cells
49
Cause of RPGN?
No specific entity
50
What is Type I RPGN? IF?
Anti-GBM GN. IF: linear ribbon-like deposits in GBM
51
What is Type II RPGN? IF?
Immune comp mediated e.g. PSGN, SLE, IgA nephropathy. IF: lumpy-bumpy, granular
52
What is Type III RPGN? IF?
Pauci-immune type. Lack of IF staining. Most have P- or C-ANCA
53
In Type III RPGN, will there be signs of vasculitis or glom damage first?
Can go either way. May have ANCA pos RPGN without signs of systemic involvement
54
RPGN gross features
Large, pale kidneys with petechiae
55
RPGN micro features
>50% of gloms with crescent formation with fibrin strands
56
RPGN EM features
Possible ruptures in GBM, with or w/o subepithelial deposits
57
RPGN clinical presentation
Nephritic: hematuria, red cell casts, mod proteinuria, HTN, edema
58
RPGN clinical course
Progressive over weeks with severe oliguria
59
RPGN treatment
Anti-GBM: plasma exchange, steroids, chemo
60
Why is there increased risk of infections + hypercoagulable state in nephrotic syndrome?
loss of Ig and complement via proteinuria, loss of anticoagulants esp anti-thrombin III
61
Most common cause of nephrotic syndrome in children
Minimal change disease
62
Most common nephrotic syndrome in adults
Focal segmental glomerulosclerosis - pts w various renal disease progress to FSGS. #2 is membranous GN
63
Secondary causes/ assocs for membranous GN
Drugs (penicillamine, captopril, gold, NSAIDs), malignancy (lung, colon, melanoma), SLE, infections, metabolic (DM, thyroiditis)
64
Pathogenesis of membranous GN
IC-mediated, (end or exogenous Ag) often phospholipase A2 receptor of podocytes
65
What is the cause of GBM damage in Membranous GN?
Complement-mediated
66
Microscopic findings in membranous GN
Normocellular gloms + uniform diffuse thickening of capillary wall (Cheerios look) + Spikes on silver stain (BM between deposits)
67
Later microscopic findings in membranous GN
Deposits turn into a very thickened GBM. Late: mesangial sclerosis, glom hyalinization
68
Membranous GN IF
Granular deposits along GBM with IgG and C3
69
Membranous GN EM
Subepithelial deposits, later spikes of BM grow between deposits, then thickened GBM + foot process effacement
70
Treatment for membranous GN
If secondary, treating cause can resolve. Otherwise corticosteroids are +/-
71
Typical course of membranous GN
Slow deterioration, chronic proteinuria
72
What is the other term for Minimal Change Disease?
Lipoid Nephrosis
73
What is minimal change disease? Peak age?
Poss T cell dysfx -> cytokines -> damage to visc epithel cells -> loss of charge barrier/ adhesion defects between epithelial cells
74
MCD associations
Atopy (eczema, rhinitis), post resp infec or routine immunization, inc incidence in Hodgkin lymphoma
75
Treatment of MCD?
Corticosteroids reverse damage to podocytes
76
Peak age for MCD
2-6 years
77
MCD micro findings
Normal gloms, prox tubules may be filled with lipid
78
MCD IF
No staining. No deposits.
79
EM in MCD
Diffuse effacement of podocyte foot processes. No deposits.
80
Clinical course of MCD
Massive proteinuria (mostly albumin), no renal failure, often no HTN
81
FSGS associations (4)
HIV, heroin addiction, sickle cell disease, morbid obesity
82
Hallmark of FSGS
Damage to visceral epithelial cells
83
What genetic abnormalities predispose to FSGS?
Muts of proteins that localize to slit diaphragm e.g. nephrin and podocin
84
Some put FSGS on a spectrum with what? Which is more severe?
MCD (less severe) -- FSGS
85
Micro findings in FSGS
Need large biopsy (focal). Collapsed GBM, inc mesangial matrix, hyalinization (Magenta w PAS) +/- foam cells
86
EM findings in FSGS
Diffuse effacement of foot processes, focal detachment of epithelial cells from GBM
87
IF in FSGS
Mesangial deposits of IgM and C3 in affected area
88
FSGS clinical presentation
Nephrotic syndrome, HTN, dec GFR, poor response to corticosteroids
89
Which renal issues recur post transplantation?
FSGS
90
HIV nephropathy most common renal complication
FSGS
91
HIV nephropathy micro findings
Cystically dilated tubules filled w proteinaceous material + inflammation
92
HIV nephropathy EM
Tubuloreticular inclusions in endothelial cells (nearly pathognomonic, also in SLE)
93
What is membranoproliferative GN?
Prolif of glomerular cells + leukocyte infiltration + GBM changes
94
Membranoproliferative GN clinical presentation
Mixed nephrotic - nephritic
95
MPGN associations (4)
SLE, HepB, HepC, malignancy
96
Microscopic appearance of MPGN (similar type I & II)
Large, hypercellular glom w lobular architecture, thickened GBM w "tram track" of mesangial cell into GBM
97
EM of Type I MPGN
Subendothelial deposits +/- supepith and mesangial

CRRAB 2 (12 decks)

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