Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Renal Week 2 > ICL 2.4: Nephritic Glomerular Diseases > Flashcards

ICL 2.4: Nephritic Glomerular Diseases Flashcards

1
Q

what does the mechanism of glomerular injury depend on?

A

the site of the antigen and pathway of complement activation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what are the different mechanisms of glomerular injury?

A
  1. fixed tissue antigens
  2. immune complex nephritis
  3. alternate complement pathway
  4. cell-mediated immune injury (cytotoxic antibodies)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the fixed tissue antigen mechanisms of glomerular injury?

A

type II hypersensitivity reaction where glomerular injury is due to antibodies to antigen
fixed to basement membrane

the antigen is fixed to the basement membrane of the capillaries in the glomeruli and antibodies come bind to the antigen which activates compliment and the compliment activation is chemotactic

antigen = non-collagen domain of collagen type IV**

ex. Goodpasture syndrome = hematuria and hemoptisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the immune complex nephritis mechanism of glomerular injury?

A

antigen and antibody circulating in the blood and then it deposits in the basement membrane to activate complement

this causes chemotaxis that calls in neutrophils, lysosomal enzymes and causes tissue damage

type III hypersensitivity reaction

ex. post-streptococcal glomerulonephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is the alternate complement pathway mechanism of glomerular injury?

A

alternate complement pathway activation starts by cleavage of C3 by bacteria or viruses or other agents

then neutrophils, enzymes and tissue damage happen

ex. membranoproliferative nephritis II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is the cell-mediated immune injury mechanism of glomerular injury?

A

T-cells damage the processes of the gomerulus

ex. minimal change disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the clinical presentation of nephrotic syndrome glomerular diseases?

A
  1. proteinuria (3+gram/24hrs)
  2. edema: when you lose protein the oncotic pressure goes down and causes edema
  3. hypoproteinemia
  4. hyperlipidemia
  5. lipiduria

lead to thickening of the basement membrane!!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the clinical presentation of nephritic syndrome glomerular disease?

A
  1. hematuria
  2. RBC casts* = structures that are molded in the renal tubules
  3. mild proteinuria
  4. HTN
  5. oliguria
  6. azotemia = high BUN and creatinine
  7. edema

leads to proliferation of the mesangial cells and necrosis = inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is the clinical presentation of hematuria syndrome glomerular diseases?

A

pure hematuria indicates variable changes in the glomeruli so we don’t really know what changes are happening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is the clinical presentation of chronic renal failure?

A

hyalinization or sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

how do you diagnose glomerular diseases?

A

① renal biopsy via light microscopy with the following special stains:

  1. PAS shows mesangium
  2. Jones silver stain shows basement membrane (important in nephrotic syndrome)
  3. trichrome shows fibrosis (important in CKD)

② then you have to save frozen tissue and do immunofluorescence –> if it’s mechanism I vs. II vs. III it’ll all look different!

③ you also need to do electron microscopy to see where the immune complexes were deposited

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are the layers of the glomerulus?

A
  1. endothelium
  2. epithelium
  3. mesangium

so the basement membrane has 3 parts = central lamina densa sandwiched between two layers called the lamina rara interna and lamina rara externa

on the inside of the glomerulus are epithelial cells and in-between them are fenestrations

then on the outside are the foot processes of the epithelial cells and in-between them are the filtration slits and diaphragm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are the 2 components of the mesangium?

A
  1. cells

they’re contractile and regulate blood flow through the glomerulus and they’re phagocytic – in some disease like post-strep, at the onset of the disease you’ll see deposits at the periphery of the capillaries but later on when the mesangial cells phagocytose the immune complexes they’ll be in the mesangium

  1. matrix

provides support

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

a 5 year old was brough tot he ED because the mother noted dark urine. the child had a sore throat 10 days earlier.

PE shows 120/80, urinarylis shows RBCs, RBC casts, 1+ protein

CBC is normal, ESR increased, elevated BUN and creatinine

diagnosis?

what other tests would you order?

A

post-streptococcal nephritis

something is effecting the kidney after a strep infection so this is an immune complex mechanism of glomerular injury

the antigen is the strep antigen! some strep antigen are still circulating and antibodies formed against it so then the classic complement pathway is activated leading to glomerular damage

ESR is high because of the inflammation happening

high BP, RBC casts, little protein, azotemia = high BUN and creatinine –> nephritic syndrome!! this means you’ll see proliferation of mesangial cells and necrosis

we want to check the complement levels which will be low since they’re being consumed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the clinical and lab presentation of post-streptococcal glomerulonephritis?

A

immune glomerulonephritis (mechanism II) = immune complex mediated nephritic syndrome = mesangial cell proliferation and necrosis

clinical presentation

  1. hematuria
  2. HTN
  3. azotemia

lab findings

  1. ASO titer increased
  2. low complement
  3. RBC in urine
  4. RBC casts in urine
  5. elevated BUN
  6. elevated creatinine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the histological, IF and EM morphology of post-streptococcal glomerulonephritis?

A
  1. mesangial cell prolifeartion and necrosis = lots of cells and neutrophils on the histology
  2. IF will show lumpy bumpby coarse granular peripheral deposits of IgG and C3 = necklace made of rocks

this is because immune complexes circulating and then they deposit in capillary membrane basement membrane

  1. EM will show subepithelial electron dense humps
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is the prognosis of post-streptococcal glomerulonephritis?

A

in children 90% resolve

in adults 70% resolve and the remainder become hypertensive and develop renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

35 year old male went to doctor for annual physical. he was found to have microscopic hematuria that wasn’t visible to the eye

CBC and Chem20 were normal

differential diagnosis?

other tests?

renal biopsy findings?

A

IgA nephropathy

but it could also be many other things like familial nephritis, stones in the kidney that just haven’t caused pain yet, tumor, etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is IgA nephropathy?

A

sometimes there’s a preceding illness like URI/GI since IgA is the mucosal antibody but there’s also familial IgA nephropathy

thought to be due to activation of alternative complement pathway by IgA

clinical findings can be either gross or microscopic hematuria

if you have hematuria the morphology can be variable so we don’t know what we’re going to see…

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is the histological, IF and EM morphology of IgA nephropathy?

A

light microscopy histology is variable because since it’s just hematuria we don’t have a hallmark thing we’re going to see –> might see crescent shape

IF will show diffuse mesangial IgA! so the deposits aren’t in the periphery, they’re in the mesangium

there is no specific EM picture

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

how do you treat IgA nephropathy?

A
  1. steroids

2. fish oil

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is the prognosis of IgA nephropathy?

A

benign in children

in adults 19% have renal failure and 32% become hypertensive –> it recurs in renal transplants**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

12 year old girl presents with a palpable red rash on her buttocks, arthralgia, abdominal pain and hematuria

UA: RBCs and RBC cell casts with increased BUN and creatinine

serum IgA

A

Henoch-Schonlein purpura

purpura = thrombocytopenia –> this is a misnomer, it’s actually a vasculitis!! there’s no thrombocytopenia

a nephritic syndrome that is a systemic disease which effects the skin, GI and kidney

palpable rash

pain without inflammation = arthralgia while arthritis is actual inflammation of the joints

IgA mediated so high serum IgA**

24
Q

what is Henoch-Schonlein purpura?

A

usually there is a preceding illness, usually a URI

  1. diffuse vasculitis
  2. transient immune complex reaction
  3. possible alternate pathway activation
  4. nephritic syndrome
  5. GI and cutaneous manifestations
  6. gross and microhematuria
  7. IgA in the kidney and skin!
  8. palpable rash**

main systems involved are the skin, GI and kidney

25
Q

what are the lab findings associated with Henoch-Schonlein purpura?

A
  1. normal platelet count and bleeding time

2. increased IgA levels in the serum

26
Q

what is the morphology associated with Henoch-Schonlein purpura?

A

LM is variable but you can have proliferation, necrosis or crescents –> proliferation can be diffuse or focal

IF will show IgA, IgG, C3, mesangial deposits, IgA deposits in the skin

27
Q

a 25 year old male presents with profuse hemoptysis following a flu-like illness a week prior

PE shows pale, tired young man

urinalysis shows many RBCs, RBC casts

CBC shows Hb of 11

differential diagnosis?

biopsy findings?

other tests?

A

Goodpasture syndrome

anemic from from coughing up blood and also blood in urine

fixed antigen to the basement membrane and then complement comes and binds to the fixed antigen which attracts neutrophils that release enzymes that cause damage

biopsy finding: the entire basement membrane is antigenic so the whole basement membrane will fluoresce!!

other tests = serologic tests to test anti-globular basement membrane antibodies

28
Q

what is Goodpasture’s syndrome?

A

fixed antigen to the kidney basement membrane

presents in the 3rd and 4th decade

often there is a preceding illness; specifically URI

clinically there will be hematuria and hemoptysis –> hematuria can precede hemoptysis or vice versa

29
Q

what is the morphology of Goodpasture’s syndrome?

A

LM shows necrosis and crescents –> Bowman’s space is obliterated and there are adhesions between the capsule and the glomerulus

IF shows linear* peripheral IgG deposits along the basement membrane of the glomerulus and the alveolar septa in the lungs

EM is not diagnostic

30
Q

what are the lab findings associated with Goodpasture’s syndrome?

A

serum anti-glomerular basement membrane (anti-GBM)

the antigen is the alpha-3 chain of type IV collagen**

31
Q

what is crescentic glomerulonephritis?

A

leakage of fibrin in Bowman’s space

so you can’t see Bowman’s space at all because it’s filled with fibrin and proliferating cells of the Bowman’s capsule = crescents!

crescents signify aggressive glomerular disease and poor prognosis = rapidly progressive –> not specifally diagnostic for any specific disease it just means a rapidly progressive renal disease

if you do IF, you’ll see an actual crescent that stains for fibrin and fibrinogen

32
Q

which disease are asccoaited with crescents?

A
  1. IgA nephropathy
  2. Henoch-Schonlein purpura
  3. Goodpasture syndrome
  4. Wegner’s
33
Q

a 45 year old female with a 3 month history of epistaxis presents with cough and hematuria?

cANCA + ve

diagnosis?

A

granulomatous polyangiitis = Wegner’s granulomatosus

with cANCA the antigen is proteinase 3 and in pANCA the antigen is myloperoxidase –> Churg Strauss (eosinophilic vasculitis)is associated with pANCA

34
Q

what is granulomatous polyangiitis?

A

previously known as Wegner’s granulomatosus

clinical findings = triad of sinusitis, pneumonitis, glomerulonephritis

bleeding from nose, lungs and kidneys

presents from 30-75 years and more in males than females

pathogenesis we don’t understand but it could be hypersensitivity or immune deficiency

35
Q

what is the morphology of granulomatous polyangiitis?

A

LM focal necrotizing lesions in the glomeruli with crescents = rapidly progressive and aggressive

IF is variable

EM shows necrosis but isn’t diagnostic

36
Q

50 year old executive presents with recurrent abdominal pain and skin rash, he has had a low grade fever for 3 months.

PE shows 140/90 BP, 100 F

urinalysis shows RBCs, RBC casts, 1+ protein

CHEM 20 shows BUN 50, creatinine 3, eosinophilia

diagnosis?

other tests?

biopsy findings?

A

polyarthritis nodosa (PAN)

could be Henoch-Schonlein purpura since there is a rash with abdominal pain BUT the rash isn’t palpable and there’s no arthralgia and this has been going on for 3 months whereas HSP is acute!

37
Q

what is polyarthritis nodosa (PAN)?

A

caused by chronic immune complex that effects kidney, GI, MSK, CNS, and skin but lungs are NOT involved***

clinical presentation depends on location, number and size of involved in vessels but most patients have fever

way more common in males than females and presents in the 4th and 6th decades

preceding illness is Hepatitis B**

38
Q

what are the lab findings associated with polyarthritis nodosa?

A
  1. ANA normal
  2. 30% will have HepB
  3. transient or persistent antigenemia from HepB
  4. positive pANC in small % of patients
  5. involves large and medium sized vessels and also the glomeruli!
39
Q

what is a crescent made of?

A
  1. fibrin
  2. parietal cells

reminds us of ARDS in the lungs which is also caused by fibrin

40
Q

platelet count in Henoch-Schonlein purpura

A

normal

it’s a vasculitis and not thrombocytopenia purpura

41
Q

IgA deposits in the skin

A

HSP

42
Q

linear deposits of IgG on IF

A

Goodpasture syndrome

43
Q

3 year old boy presents to ER with lethargy, hematuria and bloody diarrhea for 2 days following a birthday party where he ate a grilled hamburger

CBC shows Hb of 7, elevated BUN, low platelets, elevated creatinine

schistocytes on peripheral smear

diagnosis?

A

hemolytic uremic syndrome (HUS)

this is a microangiopathic hemolytic anemia!

other diseases associated with microangiopathic hemolytic anemia and renal failure are DIC and TTP –> TTP doesn’t have GI symptoms it’s just transient neurologic manifestations, microangiopathic hemolytic anemia, renal failure, thrombocytopenia and low grade fever –> DIC patients are usually septic

44
Q

what is hemolytic uremic syndrome?

A

it’s a disease where microthrombi occlude the arterioles and capillaries, which results in microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury

incidence is usually postpartum and in children under 1 year

preceding illness is almost always a gastroenteritis; specifically the toxins of E. coli

clinical presentation:
1. oliguria

  1. renal failure
  2. hemolytic anemia
  3. thrombocytopenia
  4. e. coli 0157 toxins
45
Q

which diseases are secondary glomerular diseases?

A
  1. SLE
46
Q

how is SLE a secondary glomerular disease?

A

mean age is 29 and more common females

caused by immune complexes

kidney effect = hematuria, proteinuria, casts, nephritic syndrome!!!

systemic disease that shows butterfly rash, photosensitivity,

47
Q

what are lab findings associated with SLE?

A
  1. increased ANA (screening test)
  2. anti-DNA
  3. decreased complement
48
Q

what is the morphology of SLE?

A

LM is variable

IF is full house so in the basement membrane and mesangial – you’ll also see deposits in the tubular basement membrane

EM will show subendothelial deposits** and mesangial deposits

49
Q

what are the 5 patterns of SLE?

A
  1. normal by light, electron, and immunofluorescent microscopy (class 1), which is quite rare
  2. mesangial lupus glomerulonephritis (class II)
  3. focal proliferative glomerulonephritis (class III)
  4. diffuse proliferative glomerulonephritis (class IV
  5. membranous glomerulonephritis (class V)

sclerosing changes

none of these patterns is specific for lupus

50
Q

a 45-year-old female with hypertension and pulmonary fibrosis and Raynaud’s phenomenon for 5 years presents with renal failure

ANA 1:20,000 speckled anti SCL70 +ve

she has thin leathery skin on her hands and face

diagnosis?

A

scleroderma aka progressive systemic sclerosis

progressive = doesn’t stop, it keeps progressing

systemic = kidney, blood vessels, lung, GI, skin

sclerosis = scars everywhere

51
Q

cANCA

A

Wegner granulomatis

52
Q

full house

A

SLE

53
Q

microangiopathic hemolytic anemia

A
  1. HUS
  2. DIC
  3. TTP
54
Q

HepB

A

polyarthritis nodosa = PAN

55
Q

low C3

A
  1. post-streptococcal glomerulonephritis

2. SLE

Renal Week 2 (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions