Brainscape's Knowledge GenomeTM

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


See full index

Renal > The Kidney in Systemic Disease 1/2 > Flashcards

The Kidney in Systemic Disease 1/2 Flashcards

1
Q

What are 4 vascular injury syndromes?

A

ANCA-assc glomerulonephritis
Thrombotic Microangiopathy
Lupus Nephritis
Scleroderma

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

What are the 2 pathogenesises of vascular disorder?

A
  1. inflammation of blood vessels

2. Loss of thromboresistance (= loss of normal resistance to clot formation)

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

What are the differences between medium vessel disease and small vessel disease?

A

medium vessel disease:

  • renal infarcts and distal glomerular ischemia
  • decline in GFR not assc with glomerular inflammation
  • NO RBC casts
  • usually ANCA negative
  • aka polyarteritis nodosa

small vessel disease:

  • focal necrotizing lesions with crescent formation
  • active urinary sediment
  • rapid progression of kidney failure (decline in GFR)
  • aka microscopic polyangiitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is pauci-immune glomerulonephritits? When is it usually seen?

A

negative immunefluoresence studies usually in the setting of crescentic glomerulonephritis

*often assc with ANCAs + extra renal findings (arthritis, athralgias, myalgias, fatigue) but not always and CRESCENTIC glomerulonephritis

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

What are ANCAs?

A

anti-neutrophil cytoplasmic Abs with 2 distinct patterns:

  1. C-ANCA: anti-proteinase 3
  2. P-ANCA: anti-lysosomal myeloperoxidase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What disease(s) are assc with positive C-ANCA Abs?

A

granulomatosis with polyangitis (Wegner’s)

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

What disease(s) are assc with positive P-ANCA Abs?

A
  • anti-GBM disease (~30% of pts)
  • low titers seen in SLE
  • sclerosing cholangitis, ulcerative colitis, and chron’s disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe the difference between C and P ANCAs with immunofluorescence?

A

C-ANCA: cytosplamic staining

P-ANCA: peri-nuclear staining

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

How are ANCAs pathogenic?

A

binding of ANCAs to neutrophils activates them –>
-inc contact and adhesions with endothelial cells/vascular structures by: B-2 integrin, Mac-1, Fc-gamma

  • -> endothelial cell injury (to glomeruli and blood vessels)
  • inhibition of PR-3 inactivity
  • EC activation
  • ADCC
  • complement activation via alt pathway
  • monocyte activation/release of ROS and chemotactic molecules
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

_______ cells are the primary target in small vessel vasculitis.

A

endothelial cells

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

What is a specific and sensitive test for Wegner’s Granulomatosis?

A

C-ANCA

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

What are signs and symptoms assc with granulomatosis with polyangitis (Wegner’s)?

A
  • Upper respiratory tract involvement with: rhinorrhea, sinusitism nasopharyngeal mucosal ulcerations
  • Lower Respiratory Tract involvement with: cough, dyspnea, hemoptysis, transient pulmonary infiltrates on X-ray
  • !0% have azotemia at presentation
  • fever, weight loss, arthralgias/arthritis, mononeuritis multiplex, skin lesions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

T or F: In most autoimmune mediated vascular necrosis, arteries and veins are involved

A

F: usually only arteries

**granulomatosis with polyangiitis/Weger’s has BOTH a and v!!

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

Describe the shape/type of glomerulonephritis of granulomatosis with polyangiitis/Wegners.

A

crescentric

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

What is the treatment of granulomatosis with polyangiitis?

A

cyclophosphamide based with steroids, plasmapheresis

*80-90% mortality if left untreated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the 2 types of TMAs (thrombotic microangiopathies)?

A

TTP

HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How are TTP and HUS differentiated?

A

HUS:

  • hemolytic anemia
  • renal dysfunction
  • thrombocytopenia

TTP:

  • hemolytic anemia
  • renal dysfunction
  • thrombocytopenia
  • fever
  • neurologic dysfunction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Describe the appearence of vessels on biopsy that are assc with TMAs?

A

onion skin

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

What is the pathogenesis of TMAs?

A
  • endothelial cell loses thromboresistance
  • platelet activation
  • fibrin clot formation in lumen of affected vessels
  • endothelial cell injury
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are 3 causes of endothelial damage in HUS?

A
  1. verotoxin producing E. coli that cause production of cytotoxic antiendothelial Abs
  2. chemotherapeutic agents (cyclosporine, gemcitabine, bleomycin/cisplastinum)
  3. radiation (BM transplant)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Familial TTP is seen with a genetic deficiency of …

A

ADAMTS13 (vWF cleaving protease)

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

T or F: inc levels of vWF multimers may directly enhance platelet aggregation.

A

T

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

What are the differences between TMA and DIC?

A

PT and PTT are normal in TMA and prolonged in DIC

TMAs are assc with a thrombotic diathesis
DIC are assc with a bleeding diathesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

T or F: SLE is a systemic disease that spares no organ system

A

T

25
Q

What is the ten year survival rate of SLE?

A

70%

26
Q

What are the 11 criteria for SLE?

A

mala rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, immunologic disorder, antinuclear Abs

27
Q

What are the stages of lupus nephritis?

A 
I: minimak mesangial
II: mesangial proliferative
III: focal proliferative 
IV: diffuse proliferative
V: Membranous 
VI: Advanced sclerosing
28
Q

What is the most common stage of lupus nephritis?

A

IV: diffuse proliferative

29
Q

Class __ is mild, class ___ is moderate, and class ___ is severe.

A

II, III, IV

30
Q

What pathologies can be seen in a biopsy of a class IV (diffuse proliferative) lupus nephritis?

A

wireloop lesions
hyaline thrombus (immune complex)
crescent formation
excess cells

31
Q

What can be seen in EM with a pt with lupus nephritis?

A

subendothelial and mesengial depositis

32
Q

Describe immunofluroesence pattern that is consistant with a diagnosis of lupus nephritis.

A

“full house immunofluoresence” = all 3 Ig present (IgG, IgM, and IgA) as well as C3 and C4

33
Q

What is the treatment for SLE?

A

Glucocorticoids, aspirin, and hydroxychloroquine (TLR inhibitor) are FDA approved for the treatment of lupus

*immunosuppresants can also be used (class 4 = cyclophosphamide and class 5 = mycophenolate mofetil)

34
Q

What class of lupus nephritis is assc with poor renal survival?

A

IV

35
Q

What is scleroderma?

A

disease that involves connective tissue and the microvasculature with fibrosis and vascualr occlusion

36
Q

What are the 2 subsets of scleroderma? What are they based on?

A

limited and diffuse cutaneous based on pattern of skin involvement

37
Q

What is the epidemiology of scleroderma?

A

femal predominance
more frequent in AA
1 in 4000 US adults affected

38
Q

How is renal involvement of scleroderma characterized? Renal crisis?

A

proteinuria
HYP
(mild renal dysfuction)

Renal crisis: new onset of accelerated arterial HYP and/or rapidly progressive oliguric renal failure

39
Q

How is scleroderma renal crisis treated?

A

ACE inhibitors

40
Q

What are the risk factors for scleroderma renal crisis?

A
  • early diffuse systemic sclerosis (subtype)
  • rapidly progressive skin disease
  • anti-RNA pol Abs
  • corticosteriod therapy
41
Q

What happens to the arcuate arteries with scleroderma?

A

intimal and medial proliferation with luminal narrowing

*fibrinoid necrosis and thrombus is common

42
Q

Describe the histology of interlobar arteries with scleroderma.

A

concentric sclerosing intimal thickening –> resembles onion skin

43
Q

What disease from this lecture is sinopulmonay?

A

Wegner’s

44
Q

What disease from this lecture is assc with puci-immune glomerulonephritis?

A

Wegner’s

45
Q

What is scleroderma renal crisis?

A

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

46
Q

What is the difference between microscopic polyangiitis and polyarteritis nodosa?

A

microscopic polyangiitis: small vessel disease (focal necrotizing lesions with crecentric formation) that causes glomerulonephritis (usually pauci-immune crescentric)

polyarteritis nodosa: medium vessel disease that causes macroscopic areas of ischemia and infarction –> kidney damage/dec GFR

47
Q

What is the pathogenesis of HUS?

A

Shiga toxin in circulation causes widespread endothelial injury:

  • shiga toxin binds receptor on endothelial cell
    • inc endothelin production
    • dec NO production
    • TNF released
    • toxin internalized (10% of time) –> apoptosis
  • pro-coag state–> thrombus formation
48
Q

What is the classical presentation of HUS?

A

sudden onset of irritability, lethargy, weakness, pallor, and oliguira in a SMALL CHILD, 5 - 10 days following gastroenteritis

49
Q

TTP treatment

A

plasmaphoresis

50
Q

What is the characteristic feature of lupus nephritis? (occurs in 100% of pts)

A

proteinuria

  • nephrotic syndrome in 65%
  • microscopic hematuria in 80%
51
Q

Glossary of terms to know:

inflammation of blood vessels, usually autoimmune

A

vasculitis

52
Q

autoimmune ANCA-negative medium vessel arteritis

A

Polyarteritis nodosa

53
Q

antibodies against proteinase 3 or myeloperoxidase associated with specific autoimmune diseases

A

ANCA

54
Q

autoimmune small vessel vasculitis cause of pauci-immune crescentic glomerulonephritis associated with P-ANCA

A

Microscopic polyangiitis

55
Q

autoimmune necrotizing granulomatous vasculitis of respiratory tract and cause of crescentic glomerulonephritis associated with C-ANCA

A
  1. Granulomatosis with polyangiitis (Wegener’s
56
Q

thrombotic microangiopathy of glomeruli in small children due to Shiga toxin from diarrheal E. coli H7:O157 infection

A

HUS

57
Q

thrombotic microangiopathy due to von Willebrand factor multimers from ADAMTS13 deficiency

A

TTP

58
Q

immune complex-mediated glomerulonephritis of systemic lupus erythematosus (the prototype multi-system autoimmune disease)

A

Lupus nephritis

59
Q

rapidly progressive renal failure of systemic sclerosis

A

scleroderma renal crisis

Renal (33 decks)

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