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CRRAB 2 Week 2 > Renal Path: Glomerular Disease > Flashcards

Renal Path: Glomerular Disease Flashcards

1
Q

In autosomal recessive polycycstic kidney disease, what do the kidneys lok like

A

enlarged, but SMOOTH externally. Cut sections show many small cysts in both the cortex and medulla

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2
Q

What are the 4 categories of autosomal recessive polycystic kidney disease?

A

perinatal, neonatal, infantile and juvenile. Only the last two survive infancy.

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3
Q

For those patients that survive infancy with autosomal recessive PKD, what extra-renal findings do they often show?

A

liver cysts
bile duct proliferation
congenital hepatic fibrosis (periportal fibrosis)

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4
Q

Describe medullary sponge kidney disease?

A

it’s an adult disease with multiple cystic dilatations of the collecting ducts in the medulla

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5
Q

What are the symptoms or medullary sponge kidney

A

relatively normal kidney function!!!

hematuria

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6
Q

How do children with nephronophthisis-uremic medullary cystic disease complex present?

A

polyuria and polydypsia due to a tubular defect caused by the cysts in the medulla

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7
Q

Many people on dialysis for over 10 years will get cysts which are usually asympomatic. What’s the worry with these cysts?

A

7% will develop renal cell carcinoma within the cysts

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8
Q

Describe a simple cyst - commonly found on autopsy?

A

typically cortical in location
1-5 cm in size
filled with clear fluid
single layer of cuboidal or flattened eptihelium lining
NO clinical significance except to differentiate from possible tumor.

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9
Q

On to glomerulonephritis…

What are the three histologic patterns of glomerular injury?

A
  1. hypercellularity (increased mesangial, endothelial cells, epithelial cells, WBCs, crescent formation)
  2. basement membrane thickening (BM material to deposited material)
  3. Hyalinization and sclerosis
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10
Q 
What do these terminology refer to:
diffuse
focal
global
segmental
A

diffuse - all glomeruli are affected
focal - only some glomeruli are affected
global - the entire glomerulus is affected
segmental - only part of the glomerulus is affected

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11
Q

What are the 4 basic histologic methods we use to evaluate renal disease?

A
  1. H&E
  2. Special stains: PAS, Trichrome, silver
  3. Immunofluorescence
  4. Electron microscopy
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12
Q

What color is PAS? Trichrome? silver?

A

PAS is pink - for sclerosis
trichrome is blue - cartilage?
silver - black capillary walls

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13
Q

What are the three general immune mechanisms of glomerular injury?

A
  1. antibody-mediated injury
  2. cell-mediated ijury
  3. activation of alternative complement pathway
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14
Q

What are the two kinds of antibody-mediated injury?

A

in situ immune complex deposition (antigen IN the kidney gets bound by antibody and forms a complex)

circulating immune complex deposition (antigen binds antibody elsewhere in the body and then complex gets stuck in kidney)

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15
Q

What happens in Goodpasture syndrome? WHat kind of antibody-mediated injury is this?

A

You have IgG direced against normal component sof the GBM - specifially the noncollagenous domain of the alpha 3 chain of Type IV collagen

since the antigen is in the kidney, it’s in situ immune complex deposition

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16
Q

What other organ is affected by Goodpasture?

A

lungs - same type of collagen there in the pulmonary alveoli

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17
Q

What’s the experimental rat version of goodpasture?

A

masugi

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18
Q

What histological technique can give you a goodpasture diagnosis?

A

Immunofluorescence = diffuse ribbon deposition

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19
Q

What is another version of in situ immune complex deposition where the antigen is M-type phospholipase A2 receptor? Rat model?

A

membranous (most membranous anyway)

heymann nephritis in rats

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20
Q

What’s the deposition pattern on IF in membranous?

A

granular - since the M-type phospholipase A2 receptor isn’t everywhere like GBM material is.

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21
Q

What will EM show in membranous?

A

electron dense deposits along the subepithelial aspect of the GBM

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22
Q

What is meant by the term “planted antigens”

A

Antigens are non-glomerular in origin, but localize to eh kidney and then antibodies form against them

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23
Q

What will the IF deposition pattern be for circulating immune complex nephritides?

A

granular

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24
Q

Describe progression of glomerular disease…Once GFR is reduced to 30-50%….

A

progression to end stage renal failure will proceed at a steady rate, no matter what the inciting event was

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25
Q

What two histological findings will you see after glomerular disease has progressed to ESRD>

A

focal segmental glomerulosclerosis

tubulointerstitial fibrosis

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26
Q

Describe how focal segmental glomerulosclerosis is an adaptive change?

A

When you start to lose function of the nephron, an adaptive change ocurs in the glomerulus called compensatory hypertrophy to make up for it

this leads to hemodynamic changes in individual gomeruli (including increaseed pressure)

this leads to segmental sclerosis over time with cell injury, epithelial cell loss and accumulation of proteins

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27
Q

What class of drugs can be given to hoepfully slow down the progression of focal segmental glomerulosclerosis?

A

ACE inhibitors or ARBs

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28
Q

WHy does tubulointerstitial fibrosis develop with glomerulonephritides over time?

A

As the glomeruli sclerose in FSGS, the tubule downstream from these glomeruli become ischemic

the ischemia and proteinuria are toxic to the tubule and they undergo both acute and chronic inflammation leading to scarring and fibrosis

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29
Q

Again, what are the characteristics of nephritis glomerulonephritis?

A

hamaturia with RBC cases
variable proteinuria, less than 3.5 g/d
azotemia
hypertension

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30
Q

What are the characteristics of nephrotic syndrome?

A 
proteinuria over 2.5 g/d
hypoalbuminemia
Edema
hyperlipidemia
hyperlipiduria
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31
Q

Why do you have increased infection risk in nephrotic syndrome?

A

loss of gammaglobulins in proteinuria

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32
Q

Why are you procoagulable in nephrotic syndrome?

A

loss of antithrombin III in proteinuria

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33
Q

What is the common finding in all nephrotic glomerulonephritides

A

severe damage to the podocytes

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34
Q

When does acute poststreptococcal glomerulonephritis typicall occur?

A

1-4 weeks post a strep pharyngeal or skin infection

most commonly in children, but present in all ages

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35
Q

What lab tests can help you make the diagnosis of acute poststreptococcal glomerulonephritis without doing a kidney biopsy?

A

serum C3 will be low
serum antistreptolysin O present
antiDNase B high

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36
Q

What will acute postreptococcal GN look like on plain histology?

A

large, hypercellular glomeruli with proliferation of endothelial and mesangial cells

infiltration of neutrophils and monocytes

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37
Q

What will acute poststreptococcal GN look like on IF?

EM?

A

IF will show granular IGG, IgM or C3 deposits in the mesangium and along the GBM

EM will show subepithelial humps

38
Q

What will the clinical presentation be in acute poststreptococcal GN?

A

malaise, oliguria hematuria (smoky urine!)

RBC cases, mild proteinuria, periorbital edema, mild HTN

39
Q

If you see crescents in most of the glomeruli on a slide, what does that mean?

A

rapidly progressive glomerulonephritis = bad news!

not caused by a specific entity

40
Q

What are the crescents made of?

A

proliferations of parietal epithelial cells of Bowman’s capsule mixed with inflammatory cells - obliterate the urinay space in the capsule and squish the glomerular tuft

also fibrin

41
Q

What are the classifications of rapidly proliferative GN?

A

type 1 = anti-GBM GN
Type 2 = immune complex mediated
Type 3 = pauci-immune type

42
Q

What will you see on the gross anatomy in rapidly proliferative GN?

A

large, pale kidneys with petechiae

43
Q

What will you see on microscopic evaluation in rapidly proliferative GN? EM? IF?

A

micro = screscent formation with fibrin strands
EM: ruptures in the GBM with or without depostis
IF: depends on type - positive in type 1 and 2, but negative in 3

44
Q

What is the clinical presentation of rapidly proliferative GN?

A

nephritic syndrome with hematuria with RBC casts, moderate proteinuria, HTN, edema

progressive over WEEKs instead of yers with ultimlately severe oliguria

45
Q

What is the most common cause of nephrotic syndrome in kids?

A

minimal change disease

46
Q

What is the most common cause of nephrotic syndrome in adults?

A

FSGS

followed closely by membranous

47
Q

What is the most common cause of membranous GN?

A

trick question!

85% are idiopathic

48
Q

What are the common associations/secondary causes of membranous GN?

A

drugs (penicillamine, captopril, gold, NSAIDS)

malignancy (lung, colon, melanoma)

SLE

Infections: HBV, HCV, syphilis, schistosomiasis, malaria

Metabolic disorders like DM or thyroiditis

49
Q

Describe the pathogenesis of membranous GN?

A

you get chronic antigen-antibody mediated idsease against the phospholipase A2 receptor

this leds to complement-mediated damage of the GBM

50
Q

What will you see on microscopy with HE> silver?

A

HE - normocellular gloms with uniform, diffuse thickening of capillary wall = cheerios!!

Silver stain will show “spikes” of basement membrane material laid down between deposits

over time, deposits just become part of a very thickened GBM

late, mesangial sclerosis and glomerular hyalinization

51
Q

What will IF show in membranous GN? EM?

A

IF will have granular deposits along the GBM containing IgG and C3

EM will have subepithelial deposits with spikes of basement membrane between the spikes. Eventually the deposits are resorbed and you just get a thickened GBM

also effacement of foot processes

52
Q

What is the typical course for membranous GN?

A

chronic proteinuria and slow deterioration - only 40% will develop chronic renal insufficiency and only 10 % will have renal failure and death

53
Q

What are the associations with minimal change disease?

A

atopy
may follow a respiratory infection or routine immunization
increased incidence in Hodgkin lymphoma

54
Q

What does minimal change disease respond to?

A

corticosteroids - amazing improvement!

55
Q

Describe the pathogenesis of minimal change disease?

A

it’s likely T cell dysfunction with release of cytokines that damage the viasceral epithelial cells (podocytes) leading to loss of charge barrier or adhesin between the podocytes and the GBM = effacement

56
Q

What will you see microscopically in MCD? on IF/ EM?

A

microscopid - normal glomeruli, proximal tubules may be filled with lipid

IF - no staining

EM - diffuse effacement of foot processes of the ivsceral epithelial cells, but no deposits

57
Q

What’s the clinical presentation and course of MCD?

A

MASSIVE proteinuria, which is highly selective for albumin

no renal failure and often no HTN

most respond to corticosteroid therapy rapidly but some can recur

58
Q

Back to FSGS…what are some important associations?

A 
HIV infeciton
Heroin addiction
sickle cell disease
morbid obesity
more common in african americans
59
Q

What are some ideas of why FSGS happens

A
  1. due to adaptive change in response to loss of kidney function
  2. idiopathic probably related to minimal change disease
  3. genetic abnormalities of proteins like nephrin and podocin leading to issues with slit diaphragm
60
Q

What will you see microscopically in FSGS?

A

. collapse of the GBM
increased mesangial matrix
hyalinization
with or wihtout foam cells

61
Q

What will you see on IF in FSGS? EM?

A

IF with mesangial deposits of IgM and C3 in sclerotic area

EM with diffuse effacement of foot processes and focal detachment of epithelial cells from GBM

62
Q

WHat is the clinical presentation and course of FSGS?
Steroids work?
How many will have ESRD in 10 years?
Does transplant work?
What association has the worst prognosis?

A

nephrotic syndrome
HTN and reduced GFR

poor response to corticosteroids

at least 50% will have ESRD in 10 years!

It often recurs post transplant

HIV nephropathy has worst prognosis

63
Q

What are the two things that characterize membranoproliferative GN?

A

Just read the name!

  1. proliferation of glomerular cells and leukocyte infiltration
    2 damage to the basement membrane
64
Q

What is the main association with membranoproliferative?

A

chronic immun complex disease: SLE, HBV, HCV, endocarditis, infected ventriculoatrial shunts

but also partial lipodystrophy, alpha-1 antitrypsin deficiency and malignancies like CLL, lymphoma and melanoma

65
Q

Describe the type 1 of membranoproliferative

A
  1. type 1 more common - granular deposition of C3, IgG, c1q, and C4 as subendothelial deposits
    (immune complex disease with activation of both classic and alternative complement pathway
66
Q

Describe type II membranoproliferative? What’s the other name?

A

dense deposit disease -
granular C3 deposit only!! (not IgG, C1q or C4 as in type I)

lamina densa of the GBM is ribbon-like and extremely electrno dense due to deposits of unknown material

excess activation of the alternativecompletment pathway (but not the classic!)

67
Q

What do the glomeruli look likein both Type 1 and type 2 membranoproliferative? What will silver stain do for both?

A

like flowers - super hypercellular

silver stain will show “tramtrack” or “doubl coutner” of the GBM due to mesangial icell interposition into the GBM

68
Q

What percentage will develop chornic renal fialure within 10 years with membranoproliferative?
steroids helpful? transplant?

A

50%
steroids not helpful
recurs after transplant

69
Q

What is another name for IgA nephropathy? What’s the systemic disease with overlapping features?

A

Berger Disease

Henoch-Schonlein purpura

70
Q

What is the main symptom of Berger disease?

A

recurrent hematuria - with or without proteinuria

relatively mild

71
Q

What is IgA nephropathy associated with?

A 
gluten enteropathy (celiac sprue)
liver disease (due to ineffective clearance of IgA complexes)
72
Q

What is the pathogenesis of IgA nephropathy?

A

IgA is the secretory Ig. For some reason in these people the plasma polymeric IgA is increased (unusual)

the increase leads to abberant glycosylation

the glycosylation leads to IgA immune complex formation - these complexes are deposited in the mesangium

73
Q

What will you see microscopically in IgA nephropathy?If? EM?

A

micro: variable appearance, but likely mesangial widening and proliferation

IF: mesangial deposition of IgA

EM: paramesangial and mesangial deposits

74
Q

Describe the clinical course of IgA nephropathy?

A

present with hematuria folllowing a respiratoyr, GI or urinary infection

hematuria lasta a few days, stops and recurs

variable course after presentation with 15-40% progressing to chronic renal failure over a long course - like 20 years

75
Q

What are the two major kidns of hereditary nephritides?

A

Alport syndrome

Thin membrane disease

76
Q

Describe what happens in Alport syndrome?

A 
nephritis
nerve deafness (may be mild)
eye disorders (lens dislocation, cataracts, corenal dystrophy)
77
Q

Which gender is more ofen affected by alport syndrome and why?

A

men - the most common form is x-liked dominant

but there are also autosomal recessive and autosomal dominant reported

78
Q

What is the pathology of alport syndrome in the kidney?

A

micro: glomeruli with segmental proliferation or sclerosis, persistence of fetal-like glomeruli, foam cells

EM: irregular THICK and thin GBM with splitting of the lamina densa

79
Q

What is the pathogenesis of alport syndrome?

AKA…what’s the mutation

A

defective GBM synthesis due to a mutation in the gene encoding the alpha 5 chain of type IV collagen! COL4A5

80
Q

Describe the clinical course of alport syndrome?

A

presents with hematuria in childhood or teens

iwth or without proteinuria

renal failture by age 20-50

81
Q

What is the cause of thin membrane disease?

A

abnormal genes encoding collagen chains

82
Q

What do you see on EM in thin membrane disease?

A

diffuse thinning of the GBM

83
Q

What does thin membrane disease present with? Course?

A

hematuria - almost always stays mile with an excellent prognosis

(except for hemoxygous patients - they can proress to renal failure)

84
Q

Chonric GN is the end result of specific types of GN. What will you see on the gross anatomy?

A

small, diffusely granular kidneys

85
Q

What will you see microcopically in chronic GN?

A

globally hyalinized glomeruli with atrophy and fibrosis of the tubules and interstitium

86
Q

What GN are more likely to progress to chronic GN?

A

In decreasing order….

rapidly progressive (duh)
focal glomerulosclerosis
membranous
membranoproliferative
IgA
poststreptococcal
87
Q

Nearly all lupus patients will show kidney involvement on IF how? EM?

A

IF with “full house” - stains with everything

EM with “wire loop” lesions - thickening of capillary wall by subendothelial deposits

88
Q

What age group gets henoch schonlein purpora more often? Which age group has renal abnormalitis with the HSP more often?

A

children ges 3-8 get it more often, but adults are more likely to have associated renal abnormlalities

89
Q

What percentages of diabetics will eventually has ESRD?

A

up tto 40%

90
Q

What are the general microscopid findings of diabetic nephropathy?

A
  1. capillary basement membrane thickening (hyalinization)
  2. Diffuse mesangial sclerosis
  3. Nodular glomerulosclerosis
91
Q

What happens to the GFR early in diabetes and why? What does this result in over time?

A

Because of the hyalinization of the blood vessel, hydrostatic in the glomerular capillaries increases so you actually get increased GFR early on

unfortunately this leads to glomerular hypertrophy and ultimately glomerulosclerosis

92
Q

What is a nother term for the nodular glomerulosclerosis you see with diabetic nephropathy?

A

Kimmelstiel-Wilson disease - you get hyaline masses at the periphery of the glomerulus
(associated with renal failure)

CRRAB 2 Week 2 (7 decks)

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