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Shit-list > Renal - Path > Flashcards

Renal - Path Flashcards

1
Q

1) What pt population is anti-GBM significantly more common in (men vs women, old vs young, race),
2) and why

A

1) young white males

2) men have a thicker GBM than women

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2
Q

what percentage of the capillary surface may correspond to fenestrations?

A

50%

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3
Q

w/ regards to the glomerular capillaries, what does this lack of a continuous cytoplasmic barrier facilitate? essentially, what do the fenestrations allow through and the significance?

A

fenestrations allow filtration and accessibility of macromolecules (including antibodies**) to GBM

antibodies - in diseases

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4
Q

Describe the arrangement of the cell process (pedicels) of the podocytes wrapping around the glomerular capillary

A

interdigitating - the key is that each adjacent pedicel belongs to a different cell

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5
Q

nephrotic syndrome –> “effacement” of foot processes of podocytes –> what is nichols’ “in reality” description of effacement

A

retraction of foot processes and loss of slit pore diaphragm

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6
Q

what is the result of effacement of the foot processes? (on a functional mico anatomy level, not the pathology)

A

long segments of capillary that are invested by the cytoplasm of a single podocyte

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7
Q

what else happens in addition to effacement of podocyte foot processes that actually allows the plasma proteins to leak?

A

there is detachment of foot processes from the basement membrane –> allowing the leak

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8
Q

what is the structure of the glomerular basement membrane, i.e. how it differs from most basement membranes? what does it consist of?

A

1) trilaminar structure
2) a) lamina lucida (or rara) (closer to endothelium)
b) lamina densa - double the usual thickness, double the thickness of lamina rara
c) lamina rara externa (closer to epithelial cells)

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9
Q

what does the structure of the glomerular basement membrane represent?

A

embyological fusion, at the level of the lamina densa, of 2 basement membranes - endothelial and epithelial

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10
Q

1) what constitutes the slit pore diaphragm?

2) their functions

A

1) proteins secreted by podocytes
2) a) cadherin and FAT - serve to bind adjacent pedicels
b) nephrin and podocin - play role in filtration

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11
Q

mutations in what slit pore diaphragm proteins result in congenital nephrotic syndromes? and why?

A

1) mutations in nephrin and podocin genes

2) loss of large amounts of protein in urine from defective slit pore diaphragm

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12
Q

major component of GBM

A

type IV collagen

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13
Q

3 other major components of GBM and their functions

A

1) perlecan - highly charged proteoglycan containing heparan sulfate –> imparts most of the charge properties of basement membranes (like blocking albumin)
2) entactin - glycoprotein w/ Ca binding properties
3) laminin - family of complex glycoproteins formed by 3 diff chains - impt in maintaining structure of GBM

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14
Q

what constitutes a collagen molecule (type IV)

A

3 alpha chains form a collagen molecule – there are 6 numbered alpha chains of type IV collagen - variability in composition of individual molecules - variability in basement membranes

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15
Q

most of the alpha chains are in the characteristic helical conformation, except for what part of a collagen molecule

A

the non-collagenous domain - a non-helical globular domain

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16
Q

Goodpasture syndrome

1) antibodies against what inciting antigen?
2) clinical signs/symptoms

A

1) antibodies against an epitope in the NC1 domain of the alpha3 (IV) chain
2) causes glomerulpnephritis w/ hematuria and pulmonary hemorrhage w/ hemoptysis
(Goodpasture is when they have both)

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17
Q

Mesangial cells (the mesenchymal cells of the kidney) have what properties

A

phagocytic and contractile properties

they phagocytose antibodies and Ag-Ab complexes

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18
Q

the 2 most comon types of glomerular diseases are

A

1) diabetic
2) immune-mediated
a) antibodies
b) immune complexes

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19
Q

immune complexes leading to glomerular disease can get there and cause problems by what 2 ways

A

1) deposited from circulation

2) immune complex formation in situ (ie forms in the glomerulus)

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20
Q

in situ antibodies causing glomerular disease can be against what 2 types of antigens (broad categories)

A

1) intrinsic (fixed) antigens

2) “planted” antigens originally from the bloodstream

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21
Q

large circulating immune complexes typically deposit where and why

A

1) subendothelial

2) can pass through fenestrations, but cannt pass through GBM, get stuck there

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22
Q

circulating antibodies against the GBM

1) deposit where
2) in what pattern
3) what disease do you see this in

A

1) subendothelial
2) linear pattern all along the GBM
3) Goodpasture syndrome

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23
Q

what is the treatment for anti-GBM antibodies? i.e. how do you remove them

A

plasmapheresis - anti-GBM antibodies typically circulate before being deposited - tx for Goodpasture

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24
Q

circulating antibodies against antigens in the cell membrane of podocytes

1) deposit where
2) what pathology do you see this in

A

1) deposit outside the GBM (at the level of the podocytes)

2) membranous nephropathy

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25
Q

post-streptococcal glomerulonephritis is an immune complex disease w/ antibodies against what?

A

streptococcal pyogenic exotoxin B

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26
Q

post-streptococcal glomerulonephritis - how do the immune complexes form?

A

immune complexes form partly in situ with antigens “planted” in the GBM from the circulation

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27
Q

post-streptococcal glomerulonephritis - what do you see on EM

A

subepithelial “humps”

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28
Q

post-streptococcal glomerulonephritis - what do you see on immunofluorescence

A

granular deposits - more granular than the subendothelial deposits of lupus

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29
Q

there is 1 metabolic disease of the glomerulus

1) what is it
2) what percentage of glomerular injury does it account for

A

1) diabetic glomerular injury

2) 50%

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30
Q

what is the first step in the etiology of diabetic glomerular injury

A

hyperglycemia causes non-enzymatic glycosylation of proteins in blood and in GBM

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31
Q

after glycosylation of plasma proteins and GBM proteins, what is the next step in the etiology of diabetic glomerular injury

A

some of the glycosylated plasma proteins get trapped in the GBM along w/ the glycosylated native proteins –> stims the production of new GBM protein

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32
Q

what is the eventual result of diabetic glomerular injury

A

thickened GBM - distorted by glycosylated proteins

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33
Q

diabetic glom injury - what is AGE (it is an acronym)

A

advanced glycation end-products - some of the glycosylated proteins are further metabolized, forming AGE

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34
Q

In addition to AGE what other bad fuckers are involved in fucking up your glomeruli in diabetic glomerular injury (3)

A

1) advanced oxidation protein products [AOPP],
2) the RAAS,
3) TGF-beta

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35
Q

diabetic glomerular injury - AOPP, RAAS, TGF-beta, AGE - what do they activate

A

activate/induce NADPH oxidase - produces ROS

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36
Q

diabetic glomerular injury - AOPP, RAAS, TGF-beta, AGE - activate/induce NADPH oxidase producing ROS - what does this cause (3)

A

1) mesangial matrix production
2) podocyte injury and apoptois
3) proteinuria

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37
Q

hemodynamic glomerular injury

1) what is the cause
2) etiology

A

1) HTN- hydrostatic pressure higher in glomerular capillaries than normal
2) instead of just driving filtration, it injures the glomerulus? (his notes literally say “injures them”) fucking good wording

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38
Q

hypertensive nephropathy - hemodynamic glomerular injury - the supranormal glomerular capillary pressure causes what pathologic results in the glomerulus

A

1) GBM thickening
2) mesnagial cell hypertrophy and hyperplasia
3) mesangial matrix production

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39
Q

HTN causes

1) what pathologic process
2) where (site selectivity)

A

1) hyaline sclerosis of arterioles

2) afferent arterioles but not efferent arterioles

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40
Q

HTN –> hyaline arteriolosclerosis of afferent arteriole –> causes what disease process in the glomerulus

A

gradual narrowing of the afferent arteriole lumen –> ischemic atrophy of the glomerulus

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41
Q

HTN - eventually causes what (along lines of hyaline sclerosis) and what is this term for end-stage hypertensive nephropathy

A

1) globally sclerotic glomeruli

2) ARTERIONEPHROSCLEROSIS

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42
Q

what population is arterionephrosclerosis more common in and why

A

1) African Americans - 8x more common
2) trypanosoma brucei rhodesiinse - (african sleeping sickness) - mutations in the gene for apolipoprotein L1 - does some shit that confers resistance to this disease

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43
Q

Malignant HTN - what population is it more common in and what is the typical epidemiology

A

african americans - BP > 200/120, causes HA, vomiting, proteinuria, hematuria, scotomas (“spots before the eyes”) and renal failure –> youngish black males around age 40

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44
Q

Malignant HTN produces what disease process of the arterioles and what does this lead to in the glomeruli

A

fibrinoid necrosis of the arterioles –> leads to necrosis of glomeruli

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45
Q

what other buzz word process does malignant HTN cause in small arteries

A

“onion skinning” - proliferation of intimal cells in small arteris - hyperplastic arterosclerosis

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46
Q

how specific are hyperplastic arteriosclerosis and arterial fibrinoid necrosis for malignant HTN

A

not very

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47
Q

small arteries and arerioles damaged by malignant HTN can do what, causing what appearance of the kidney

A

burst - cause “flea-bitten” kidney

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48
Q

Malignant HTN is what (big red slide, do not miss dx)

A

MEDICAL EMERGENCY - it can be fatal - 5 yr survival is only 50%

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49
Q

what is the most common cause of death in malignant HTN

A

renal disease - 90%

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50
Q

it is unique to kidney biopsies to require what 3 types of microscopy

A

1) light
2) immunofluorescence
3) electron

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51
Q

Diffuse

A

involving all or most of the glomeruli

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52
Q

focal

A

involving some but not most of the glomeruli

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53
Q

Global

A

involving the whole glomerulus

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54
Q

Segmental

A

involving only part of the glomerulus

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55
Q

Proliferative

A

increased cells - proliferating native cells, but also infiltrating inflammatory cells

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56
Q

membranous

A

increased GBM - w/out increased cells

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57
Q

MPGN

A

membranoproliferative (glomerulonephritis) combination

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58
Q

crescentric (glomerulonephritis)

A

disease involving Bowman space with proliferating parietal cells and infiltrating macrophages

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59
Q

necrotizing w/ organizing

A

has some repair processes going on

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60
Q

“pauci-immune”

A

ANCA antibodies involved in crescentic glomerulonephritis but these antibodies are not visible on immunofluorescnce or electron microscopy of glomeruli

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61
Q

glomerulosclerois

A

fibrous scar replacing glomerulus

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62
Q

“acute necrotizing crescentic glomerulonephritis”

A

infiltration of immune cells, mostly macrophages, less commonly neutrophils, causing cell death quickly. Crescentic = Bowman’s space - inflammation of - parietal epithelial cells proliferate into the open space

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63
Q

crescentic is…

A

literally. the worst

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64
Q

“focal segmental glomerulosclerosis”

A

scarring of parts of a few of the glomeruli - more advanced than acute

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65
Q

prognosis of crescentic glomerulonephritis

A

bad

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66
Q

Post-infectious glomerulonephritis (PIGN) - location of deposits

1) early in the disease
2) later in the disease
3) the significance

A

1) sub-endothelial
2) sub-epithelial
3) most pts don’t get biopsied until later in the disease course - when deposits are sub-epithelial

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67
Q

for immune complex deposition diseases of glomerulus, what other site of deposition is usually associated with subendothelial deposition

A

mesangium

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68
Q

what 2 diseases are characterized by subepithelial deposits

A

1) post-infectious GN

2) membranous nephropathy

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69
Q

what is a characteristic feature of the antigen associated with post infectious GN

A

it is planted there - immune complex forms in situ

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70
Q

what is a characteristic feature of the antigen associated with membranous nephropathy? what pattern of deposition does it lead to?

A

1) antigen is intrinsic to the podocytes

2) deposits are continuous along the subepithelial space

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71
Q

anti-GBM disease

A

Goodpasture

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72
Q

mesangial deposits typical of which pathology

A

IgA nephropathy

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73
Q

what is the pattern associated with Anti-GBM ab binding

A

Abs bind in a linear fashion

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74
Q

(in general, as a clinician) when you see a pt come in w/ edema, what 3 pathologies should you think of?

A

1) CHF
2) cirrhosis
3) proteinuria = nephrotic syndrome

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75
Q

what are the 3 purposes/ indications for kidney biopsy

A

1) Dx
2) prognosis
3) Guide therapy
“guiding therapy or elucidating a failure to respond to therapy is an indication for biopsy”

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76
Q

recall the 3 slide preparations that are unique to kidney biopsy (no other tissue sample requires all three of these)

A

1) light microscopy
2) immunofluorescence
3) electron microscopy

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77
Q

what are some of the requirements of all kidney biopsies

A

1) each slide prep requires a different tissue processing
2) all require rapid placement into appropriate preservative
3) all require planning and coordination to have the right reagents on hand and the right transport carried out

i.e. do not do them at 5 PM on a Friday afternoon

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78
Q

what are the absolute and relative contraindications to kidney biopsy? (5)

A 
absolute 
1) bleeding diathesis 
2) uncontrolled HTN
relative 
3) single kidney 
4) high pressure hydronephrosis
5) adult polycystic kidney disease
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79
Q

buzz word “foot process effacement”

seen on EM

A

minimal change disease (MCD)

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80
Q

buzz word “spike and dome”

seen on EM

A

membranous nephropathy

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81
Q

buzz word “subepithelial humps”

seen on EM

A

post-infectious glomerulonephritis

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82
Q

what is Larry’s new, favorite word instead of “effacement”

A

“fusion” - fusion of foot processes

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83
Q

buzz word “tram tracks”

A

membranoproliferative glomerulonephritis

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84
Q

buzz word “basketweave”

A

alport syndrome

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85
Q

buzz word “wire loops”

A

lupus nephritis

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86
Q

buzz word “onion-skin”

A

HTN nephropathy or scleroderma

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87
Q

what particles are the most easily filtered through the glomerular barrier?

A

small size, cationic particles

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88
Q

what is the size barrier? (2)

A

lamina densa of GBM and the slit diaphragms

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89
Q

what is the charge barrier?

A

lamina rara interna, and apparently the fenestrated capillary endothelium

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90
Q

dysfunction of alpha-Actinin 4 causes what

A

AD FSGS

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91
Q

dysfunction of podocin

A

AR steroid resistant FSGS

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92
Q

dysfunction of TRPC6

A

gain of fxn mutation –> AD FSGS

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93
Q

dysfunction of NEPH1

A

AR Finnish type, resembles MCD

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94
Q

what example of a low molecular weight protein did we mention as one that has almost completely unrestricted filtration through GBM

A

beta2 microglobulin

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95
Q

what happens to the protein that does get filtered and arrives in the tubular lumen?

A

almost all gets reabsorbed in the PCT, only a small amt gets excreted in urine

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96
Q

what is the mechanism of protein reabsorption in the PT

A

endocytosis - by the epithelial cells in the PT

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97
Q

amount of protein that reaches Bowman’s space is a direct function of what? and what clinical significance does this have?

A

1) intraglomerular pressure

2) this is a target for anti-HTN meds –> a means to reduce proteinuria

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98
Q

compare the filtration of both small and large size proteins in normal kidney function and NS

A

1) large proteins are filtered more easily in NS
2) small proteins are filtered more easily in nml GBM function, decreased filtration in NS - result of less surface area available for filtration w/ NS

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99
Q

what is the lowest amount of albumin that can be picked up by a urine dipstick? what is the sensitivity of the dipstick?

A

300 mg/day

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100
Q

what is the normal amt of albumin excreted daily

A

30 mg/day

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101
Q

how do you measure albumin levels of between 30-300 mg/day

A

spot ratio test - find the ratio of urine albumin/creatinine OR can use the microalbuminuria

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102
Q

which test is preferred between the spot ratio albumin/creatinine or the microalbuminuria for detecting albumin in urine 30-300 mg/day

A

the spot ratio test - corresponds fairly accurately to the proteins collected in a 24 hr urine collection - means we don’t have to run 24 hr collections much anymore

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103
Q

what is a normal 24-hr urine collection level of albumin

A

less than 150 mg

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104
Q

what is the normal spot urine creatinine ratio

A

less than 0.15

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105
Q

what is the nephrotic range of 24-hr urine collection

A

greater than 3.5 grams

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106
Q

what is the nephrotic range for spot urine protein creatinine ratio

A

greater than 3.5

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107
Q

For minimal change disease, what is the pathological appearance on light microscopy?

A

normal

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108
Q

Minimal Change Disease, what 2 main pathologic appearances are we looking for on Electron Microscopy?

A

1) effacement/fusion of foot processes

2) detachment of foot processes

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109
Q

Why is it relatively easy to miss the Dx of FSGS? and if so, what will you think/treat it as/ what will you see?

A

1) if biopsy misses an area of scarring (b/c it is focal and segmental) then will miss dx
2) MCD - will see poor response to steroid therapy

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110
Q

What percentage of pts w/ FSGS develop end stage kidney disease? what is the time frame?

A

50% of pts w. FSGS develop end-stage kidney disease w/in 10 yrs of diagnosis

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111
Q

what is the novel discovery in primary FSGS?

A

suPAR - soluble urokinase-type plasminogen activator receptor - associated w/ FSGS, permeability facotr released by T cells

112
Q

what does suPAR bind and where?

A

in the glomerulus, suPAR binds w/ integrin - leads to dysfunction of podocytes

113
Q

what chromosome is the apolipoprotein L1 gene (APOL1) located on?

A

22

114
Q

what is the most common cause of nephrotic syndrome in african American adults?

A

FSGS

115
Q

the most common cause of nephrotic syndrome in white adults

A

membranous nephropathy

116
Q

adhesion of involved segment to Bowman capsule is an important feature of what disease

A

FSGS

117
Q

which subtype of FSGS has the worst renal survival?

A

collapsing

118
Q

which subtype of FSGS is more likely to obtain remission (best prognosis)?

A

Tip

119
Q

What did Larry make a point to say about the Tip variant of FSGS?

A

it is very focalized and it is on the opposite pole

120
Q

What was Larry’s epidemiology example of collapsing FSGS

A

HIV autopsies from LA county in the 80s (he likes it - probably on test)

121
Q

what is the most common tx for FSGS (initially?)

A

corticosteroids

122
Q

If a FSGS pt is non responsive to corticosteroids, what is the 2nd line treatment option? What was Larry’s point about this tx option?

A

calcineurin inhibitors (cyclosporine, tacrolimus) - they themselves are nephrotoxic

123
Q

if a pt is 50 yr or older, present w/ membranous nephropathy, what do you need to immediately check them for?

A

look for tumor as a cause of secondary MN

124
Q

what % of MN spontaneously resolves?

A

30%

125
Q

what % of MN progresses to renal failure?

A

40%

126
Q

Since 30% of MN spontaneously resolves, how does this guide our tx?

A

Rx for MN is selective - only provided for pts who are at high risk for progression to ESRD -i.e. you don’t need to treat everyone w/ MN w/ cytotoxic agents

127
Q

for MN, what are the 6 risk factors for loss of renal function?

A

1) male
2) 10 g/24 h proteinuria
3) HTN
4) Azotemia
5) Tubulointerstital fibrosis
6) glomerulosclerosis

128
Q

for MN - what is our deciding factor for whether to tx w/ supportive measures or w/ disease modifying?

A

proteinuria

less than 4 gm - supportive

more than 4 gm - disease modifying

129
Q

membranoproliferative glomerulpnephritis can be associated with which type of gammopathy, monoclonal or polyclonal? what will you see on immunofluorsecence

A

monoclonal gammopathy

immunofluorescence - the Ig will be exclusively kappa or lambda - only one will light up

130
Q

what is the classic appearance of dense deposit disease on EM

A

dense dark continuous ribbons

131
Q

what is the most common primary glomerulonephritis world wide?

A

IgA nephropathy

132
Q

what is the tx like for Goodpasture?

A

aggressive - “kitchen sink” - corticosteroids, plasmapheresis, and cytotoxic agents

133
Q

from Larry - in Anti-GBM disease, in addition to cresecents, what will you see on EM

A

breaks in the basement membrane

134
Q

young male p/w hematuria and a fmaily hx of renal problems - what is most likely

A

Alport

135
Q

what is another associated symptom w/ alport

A

sensorineural hearing loss

136
Q

Alport - “basket weave” - what does this refer to?

A

the splitting of the lamina densa

137
Q

medium vessel disease - renal infarcts; is this associated with glomerular inflammation and RBC casts? ANCA status?

A

1) not associated w. glomerular inflammation w/ RBC casts

2) ANCA negative (Think PAN)

138
Q

small vessel disease - crescent formation; what do we see in UA? progression of kidney failure? ANCA?

A

1) active urinary sediment
2) rapid progression of kidney failure
3) ANCA positive

139
Q

pt comes in, mid 60s, ANCA negative, maybe Hep B or C, fatigue, wt loss, weakness, fever, arthralgias - what you thinking?

A

Polyarteritis nodosa

140
Q

PAN w/ Hep B - what else are you likely to see w/ regards to the kidney

A

membranous nephropathy

141
Q

PAN w/ Hep C - what are you likely to see w/ the kidney

A

MPGN

142
Q

PAN - characteristics of vasculitis

A

segmental transmural necrotizing

143
Q

PAN - what will you see in histo for

1) early in the disease
2) later

A

1) early = neutrophils

2) fibrinoid change, necrosis of vessel wall

144
Q

what is a key physical exam finding for PAN that is also a way to remember the pathology?

A

palpable subcutaneous nodules - they are thrombosed or severely inflammed aneurysms = “nodosa”

145
Q

What are 2 pathologic processes that can be seen w/ PAN

A

1) renal infarcts

2) arterial aneurysms

146
Q

what is a bad, silent complication of PAN

A

if an aneurysm in the kidney bursts - retroperitoneal hemorrhage, pt p/w hypotension and syncope

147
Q

Binding of ANCAs to neutrophils results in PMN activation - what does this lead to

A

increased contact and adhesion w/ ednothelial cells and vascular structures

148
Q

Binding of ANCAs to neutrophils results in PMN activation - adhesion to endothelial cells and vascular structures - what moderates the adhesion (3)

A

1) Beta-2 integrin
2) Mac-1
3) Fc gamma

149
Q

what is significant about ANCAs in small vessel vasculitis, and how does this relate to treatment

A

ANCAs are pathogenic - we can treat by plasmapheresis

150
Q

Granulomatosis w/ polyangiitis - what kind of syndrome is it (think about where it all affects)

A

sinopulmonary renal syndrome

151
Q

Granulomatosis w/ polyangiitis - in addition to the sinus/pharynx and lower respiratory tract symptoms, what else may be seen

A

fever, wt loss, arthralgias/arthritis mononeuritis multiplex, skin lesions (papules, vesicles, purpura)

152
Q

histo slide - multinucleated giant cells (subtle), pathergic necrosis, blue haze

A

granulomatosis w/ polyangiitis

blue haze = nuclear dust from breakdown of polys

153
Q

granulomatosis w/ polyangiitis - what kind of GN

A

crescentic

154
Q

hemolytic anemia (schistocytes on blood smear), renal dysfunction, thrombocytopenia (due to platelet consumption)

A

Hemolytic uremic syndrome

classic clinical triad

155
Q

hemolytic anemia, thrombocytopenia, renal dysfunction + fever and neurologic dysfunction

A

Thrombotic thrombocytopenic purpura

156
Q

what is the first step of pathogensis of thrombotic microangiopathy

A

loss of thromboresistance by the endothelial cell

157
Q

what is the subsequent pathogenesis of thrombotic microangipathy

A

platelet activation - deposition of platelet and fibrin thrombi in lumen of affected vessels

158
Q

where else may fibrin deposit in thrombotic microangiopathy

A

subintima and media of vessels

159
Q

classic, but nonspecific appearance of vessel on biopsy of thrombotic microangiopathy

A

onion skinning (can also be HTN, etc)

160
Q

thrombotic microangiopathy - what 3 things can cause endothelial damage

A

1) verotoxin producing E coli H7:O157 - may cause cytotoxic antiendothelial antibodies
2) chemo agents (cyclosporine, gemcitabine, bleomycin, cisplatin)
3) radiation - bone marrow transplant

161
Q

TTP - ADAMTS13 - if this is acquired

1) what is pathogenesis
2) what is tx

A

1) autoantibodies to ADAMTS13

2) plasmapheresis

162
Q

TMA vs DIC -

1) PT and PTT
2) diathesis (bleeding or thrombotic)

A

TMA

1) PT and PTT nml
2) thrombotic diathesis

DIC

1) PT and PTT elevated
2) bleeding diathesis

163
Q

what renal disorder is seen w/ lupus

A

1) proteinuria

2) or - cellular casts - RBC, Hgb, granular, tubular, or mixed

164
Q

Class I Lupus nephritis - pathogensis

A

minimal mesangial

165
Q

Class II Lupus

A

mesangial proliferative - 15% - mild

166
Q

Class III Lupus

A

Focal proliferative - 25% - moderate

167
Q

Class IV Lupus

A

diffuse proliferative - 50% - severe

168
Q

Class V Lupus

A

Membranous - 10%

169
Q

Class VI Lupus

A

Advanced sclerosing

170
Q

Lupus Class IV - histo features

A

lost of excess cells - inflammatory cells, mesangial cells, cpaillary endothelial cells - wireloop lesions, hyaline thrombus, crescent formation

171
Q

Lupus V - rare, but what is a defining feature

A

nephrotic

172
Q

Lupus - immunofluorescence - what all is present in deposits

A

Full house - IgG, IgM, IgA, C3 and C4

173
Q

what tx of Lupus do you worry about adherence w/ (oral) and why

A

1) Mycophenolate mofetil

2) GI side effects

174
Q

Mycophenolate Mofetil MOA

A

inhibition of Toll-like receptor

175
Q

what are the 2 subtypes of scleroderma and which one are you more worried about for renal involvement

A

1) Diffuse cutaneous - more of a concern for renal involvement
2) limited cutaneous

176
Q

scleroderma renal involvement - what is the manifestation

A

renal dysfunction, proteinuria, HTN

177
Q

what is the tx of scleroderma renal crisis and what is unique about it

A

1) ACE I - highest tolerable level
2) scleroderma renal crisis is the one scenario where you will raise the ACE I as high as possible in the setting of renal dysfunction

178
Q

what are the 4 risk factors for scleroderma renal crisis

A

1) early diffuse systemic sclerosis
2) rapidly progressive skin disease
3) anti-RNA polymerase antibdies
4) corticosteroid therapy

179
Q

what renal arteries are affected in scleroderma and what is the manifestation

A

1) arcuate arteries

2) intimal and medial proliferation w/ luminal narrowing

180
Q

scleroderma -

1) what arteries (besides arcuate) are affected
2) manifestation, what is the common slang

A

1) interlobar arteries
2) concentric sclerosing intimal thickening - “onion skinning” - of bigger arteries (150-200 microns d) than other diseases we have talked about

181
Q

what is the definition of scleroderma renal crisis

A

new onset of accelerated arterial HTN and/or rapidly progressive oliguric renal failure

182
Q

what subset of AKI is the worst cases

A

ATN

183
Q

what % of ATN does ischemic ATN comprise

A

iscehmic ATN is around 75% of all the cases of ATN

184
Q

3 gross pathologic features of ATN

1) size of kidney
2) cortex
3) medulla

A

1) enlarged - up to 30% over nml
2) pale cortex
3) congested medulla - especially at corticomedullary junction

185
Q

for ischemic ATN, what is the most common cause for the ischemia?

A

shock, especially septic shock

186
Q

what is a common gross kidney feature of ATN and what is the cause

A

1) cortical hemorrhages

2) septic emobli - somehow - something that unevenly affects the kidney

187
Q

ATN - what are 2 features seen on histo associated with early ischemic changes

A

1) blebbing on the luminal side of the tubular epithelial cells - replace the brush border
2) vacuolization of cytoplasm - diffuse edema of tubular cells

188
Q

ATN - what are 4 microscopic patho findings that are not the early 2

A

1) flattening of epithelium
2) necrosis
3) sloughing of epithelial cells into lumen
4) cells mixing into proteinaceous casts in lumen (“muddy brown”)

189
Q

ATN - what is another, very rare, feature seen on histo in addition to / opposed to necrosis

A

apoptosis of few tubular epithelial cells

190
Q

ATN - histo - what can show up as dense, acellular, darkly eosinophilic casts in tubules

A

myoglobin -

191
Q

ATN - what / how does myoglobin fit into the patho picture

A

crush injury or trauma - rhabdo - release of myoglobin

the crush injury or trauma can also lead to shock which leads to ATN

192
Q

ATN - see prominent cytoplasmic vacuolization and formation of oxalate crystals in tubular lumen - what is etiology

A

ethylene glycol poisoning

193
Q

ATN - what is seen in recovery phase

A

mitosis

194
Q

when does ATN become a medical emergency? what is the number?

A

hyperkalemia - K over 7 mmol/L

195
Q

what is the tx of hyperkalemia

A

1) IV calcium gluconate - antagonizes membrane depolarization - guards against cardiac arrhythmias
2) IV insulin + glucose - drives potassium back into cells

196
Q

Acute pyelonephritis - gross patho - dark red congestion and areas of light tan suppurative inflammation - what feature can be present in addition to necrosis

A

coalescing into abscesses

know that acute pyelonephritis is abscessing (liquefactive necrosis)

197
Q

pyonephrosis

A

pus replacing the kidney

198
Q

perinephric abscess

A

abscess extending into adjacent tissue

199
Q

pyelonephritis can extend out to capsule - causes necrosis - then what does it cause? what is characteristic feature of this?

A

1) cortical scar, depressed inwards

2) blunted calyx

200
Q

what are 2 routes of cause of pyelonephritis and which is more common

A

1) ascending infection from the bladder (E coli) - most common
2) hematogenous spread (staph aureus)

201
Q

what is the pattern of pyelonephritis associated w/ ascending

A

large area pyelonephritis - almost wedge shaped

202
Q

pattern of pyelonephritis associated w/ hematogenous

A

foci of pyelonephritis - usually infection of individual glomeruli

203
Q

chronic pyelonephritis - what inflammatory cells present and where do they land

A

1) lymphocytes and plasma cells

2) primarily interstitial

204
Q

chronic pyelonephritis - buzzword

and cause

A

1) thyroidization

2) fibrosis and tubules distended w/ inspissated urine - make them look like thyroid follicles

205
Q

most common cause and % of acute interstitial nephritis (AIN)

A

drug reactions

75%

206
Q

AIN - in addition to usual inflammatory cell infiltrate (macs, lymphos, neutros) what else is characteristic of histo

A

edema

eosinophils (least common, most specific)

207
Q

Analgesic nephropathy - what is the pathophys leading to papillary necrosis

A

chronic interstitial nephritis - corticomedullary junction - restricts blood flow to medulla - some liquefactive necrosis takes place - leads to ischemic necrosis of papillary tip - then sloughing off

208
Q

ADPKD - mutation in what protein is most common cause

A

polycystin - 1

209
Q

ADPKD - pathogensis

A

ciliopathy (due to defective polycystin) - defective mechnosensing of urine flow, dysregulation of cell adhesion

210
Q

ARPKD - (children) - mutation in what protein

A

fibrocystin

211
Q

what is sequalae of ARPKD

A

immediate untreatable respiratory failure at birth - pulmonary hypoplasia - b/c fetal kidneys do not produce enough amniotic fluid

212
Q

ARPKD - where do cysts form initially

A

down in collecting duct

213
Q

what is the most common genetic cause of ESRD in children?

A

nephronophthisis - medullary cystic disease complex

214
Q

what is nephronophthisis

A

small kidneys w/ numerous small cysts at corticomedullary junction and chronic tubulointerstital nephritis and fibrosis

215
Q

nephronophthisis - what are the mutations

A

9+ genes for cilia components

216
Q

medullary sponge kidney

A

“relatively common and usually innocuous condition” - nothing, compared to medullary cystic disease complex

217
Q

recall: what biochem feature causes sickle cell disease

A

substitution of a valine for glutamic acid as the 6h amino acid of the beta globin chain - Hgb tetramer is poorly soluble when deoxygenated

218
Q

Clinical manifestations of sickle cell

A

PAINFUL EPISODES due to hemolysis, symptomatic anemia, infetions, stroke, etc
(priapism)

219
Q

PBS of Sickle Cell

A

sickle cells, polychromasia (inc retics), Howell-Jolly bodies - hyposplenia, possible target cells

220
Q

impt lab findings for Sickle Cell

A

inc retic, hyperbilirubinemia, elevated serum LDH, low serum haptoglobin

221
Q

what will you see in sickle cell pt that indicates renal involvement

A

microalbuminuria - 60% of patients w/ hgb SS disease those over 45 years

222
Q

what percentage of pts w/ Hgb SS disease will develop end stage renal disease

A

4-12%

223
Q

What 3 features of (blank) part of the kidney cause a perfect storm for formation of sickles

A

(blank) = inner medulla
1) hypoxia - partial pressur of O2 10-35 mm Hg
2) acidosis
3) hyperosmolarity

224
Q

what happens to renal vasculature in sickle cell trait

A

the cortical vasculature is decreased; vasa recta attenuated

225
Q

what happens to renal vasculature in sickel cell disease

A

considerable decrease in cortical vasculature and vasa recta completely obliterated

226
Q

What are the two main clinical findings of sickle cell kidney problems?

A

1) hematuria

2) hyposthenuria (loss of concentrating ability of nephron)

227
Q

what is the clinical finding in early course of sickle cell renal problems

A

initially increased GFR

manifest as lower creatinine than expected

228
Q

sickle cell renal involvement - what are the other features of shit going on in nephron

A

1) microalbuminuria - proteinuria
2) supranormal PT function - hyperphosphatemia and inc Cr secretion
3) impairment of distal hydrogen ion and K secretion

229
Q

early stages of sickle cell npehropathy - what will you see on histo

A

glomerular hypertorphy
hemosiderin deposits
focal areas of hemorrhage or necrosis

230
Q

later stages of sickle cell nephropathy - what will you see on histo

A

edema, fibrosis, tubular atrophy (bad news bears) and papillary infarcts – papillary necrosis (impt)

231
Q

end stage of sickle cell nephroapthy

A

FSGS w/ glomerular enlargement (more hypertrophy than MPGN)

232
Q

AL amyloid - aka primary type - key feature of the immunoglobulins that are present

A

monoclonal

233
Q

AL (primary) - when circulate through the kidney what makes it to urine

A

free light chain - Bence Jones Proteins

234
Q

2 most common disease in US that lead to secondary (reactive - AA) type amyloidosis

A

1) Rheumatoid Arthritis

2) inflammatory bowel disease

235
Q

what has to present in the extracellular environment to allow for amyloidosis to occur

A

extracellular fibrillogenic environment w/ amyloid enhancing factor secreted by macrophages

236
Q

amyloidosis - what is key shape of the proteins and what is the importance of this

A

beta pleated sheets

macrophages cannot clear the proteins in this conformation

237
Q

amyloidosis in the kidney - what are the 2 results of amyloid deposits that lead to poor kidney function

A

1) tubular atrophy

2) interstitial fibrosis

238
Q

what is a setback of the congo red stain

A

false negatives - looks peachy in color or more salmon (some might say it almost looks taupe)

239
Q

what is immunoperoxidase stain used for and what is a potential setback

A

1) diff type of amyloid

2) often false positives

240
Q

what is a key associaiton with Amyloid L (i.e. cause)

A

monoclonal gammopathy - b/c immunoglobulin light chains are usually monoclonal (although multiple myeloma can cause, less frequently)

241
Q

AL associated w/ diffuse symptoms overall -

1) BP
2) heart
3) GI
4) blood

A

1) not HTN
2) restrictive cardiomyopathy, sick sinus syndrome
3) GI dysmotility, malabsorption, hemorrhage or obstruction
4) bleeding diathesis

242
Q

what can happen when Bence Jones proteins preceipitate in th tubules and what does this look like

A

1) tubular epithelial cells coalesce into a syncytium around the BJ proteins
2) looks like a multinucleated giant cell

243
Q

what is the tx for AL

A

1) low dose melphalan and dexamethasone

2) high dose melphalan and autologous stem cell transplant in patients

244
Q

AA (reactive systemic) amyloidosis - what is third cause, in addition of RA and IBD

A

ankylosing spondlyitis

245
Q

AA produced in liver in response to what

A

IL-1 and IL-6

246
Q

AA kidney is the main target organ - what is the most common COD

A

pts end up on dialysis - most common COD is infection from dialysis related thing

247
Q

what is treatment for AA

A

eprodisate - limits deposition of amyloid A fibrils by interfering w/ their interaction w/ tissue glycosaminoglycans
slows renal decline but not risk of death

248
Q

what is cause of amyloid Beta2m

A

long-term hemodialysis

249
Q

where does amyloid Beta2m commonly deposit (this is impt)

A

synovium, joints, tendon sheaths

250
Q

amyloid TTR - what is function of transthyretin

A

transports thyroxine and retinol

251
Q

amyloid TTR - what are presenting complications

A

heart failure or arrhythmia

commonly heart block - deposits in conducting system, blocking a bundle branch or something

252
Q

what disease is commonly associated w/ multiple myeloma

A

light chain deposition disease

253
Q

light chain deposition disease - what doe we call it

A

a nonamyloid monoclonal immunoglobulin deposition disease (MIDD)

254
Q

light chain dis (MIDD) - obviously congo red negative, what else are features (microscopic) of this

A

no fibrillar organization - precipitation of immunoglobulin chains without elongation seen in amyloid

255
Q

sicca syndrome - what are thinking

A

light chain deposition disease

sicca - dry eyes, dry mouth

256
Q

general symptoms fo light chain deposition disease

A

proteinuria w/ renal failure, hepatomegaly, cardiomegaly, CHF, conduction disturbances, peripheral neuropathy, GI distrubances, pulmonary nodules, arthropathy, (similar to amyloidosis)

257
Q

features of light chain disease on histo slide 3)

A

1) nodular glomerulosclerosis
2) expansion of mesangial matrix
3) deposits may be seen along the outer part of tubular basement membrane
strongly resembles diabetic nephropathy

258
Q

Light chain disease on IF - features

and kappa or lambda more common

A

1) light up on capillary loops, mesangium and noted tubule involvemnt
2) kappa

259
Q

light chain disease - EM

A

endothelial cell side, looks like silk

260
Q

pt p/w nephrotic or RPGN, cardiac diastolic dysfunction, and monoclonal Ig in urine or serum

A

Light Chain disease - will see proteinuria, will need to identify (chemical analysis) the protein to determine monoclonal Ig

261
Q

MIDD - renal and patient survival better w/ what?

A

if no cast nephropathy, better survival

262
Q

pt has heavy proteinuria w/ minimal swelling and progressive azotemia - what are thinking

A

HIVAN

263
Q

HIVAN - buzz word of what you will see on light microscope histo

A

microcystic tubular dilatation

264
Q

HIVAN - what else seen on histo (3)

A

1) collapsing form of FSGS,
2) intersitial inflammation and fibrosis
3) podocyte dedifferentiation and proliferation

265
Q

HIVAN - what is a key susceptibility allele and what pt population is this in

A

APOL1

African Americans - show increased susceptiblity to HIVAN and other renal disease

266
Q

what tx can be used in HIVAN w/ only mild renal dysfunction

A

ACEI or ARB (should not be used in pts w/ advanced severe renal dysfunction)

267
Q

again, what are 2 features we see on histo for HIVAN

A

1) collapsing FSGS

2) microcystic tubular dilation w/ interstitial inflammation and fibrosis

268
Q

what is the patho of mixed cryoglobulinemia type II

A

B-cell lymphoproliferative disorder characterized by the deposition of immune ocmplexes

269
Q

mixed cryoglobulinemia type II - what do the immune complexes contain (4)

A

rheumatoid factor (RF), IgG, HCV RNA, and complement

270
Q

mixed cryoglobulinemia type II - where do immune complexes deposit and what does it cause

A

endothelial surfaces - elicits vascular inflammation (hematuria)

271
Q

what kind of renal disease picture does cryoglobulinemia lead to

A

MPGN (type 1?)

272
Q

Meltzer’s triad of purpura, arthralgia, and weakness - what disease

A

cryoglobulinemia

273
Q

most common symptoms of cryo

A

cutaneous manifestations - erythematoous macules to purpuric papules

274
Q

what type of Ig is RF

A

IgM - binds IgG

275
Q

what type of glomerulopathy is amyloidosis

A

nonproliferative, noninflammatory

276
Q

what do we see on EM for amyloidosis

A

randomly oriented fibrils

Shit-list (2 decks)

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